635 results on '"Birbeck granules"'
Search Results
2. Orbital Histiocytic Disease
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Verity, David H., Vahdani, Kaveh, Rose, Geoffrey E., Steele, Eric, Section editor, Ng, John, Section editor, Albert, Daniel M., editor, Miller, Joan W., editor, Azar, Dimitri T., editor, and Young, Lucy H., editor
- Published
- 2022
- Full Text
- View/download PDF
3. Indeterminate Cell Tumor
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Rezk, Sherif A, Spagnolo, Dominic V, Brynes, Russell K, and Weiss, Lawrence M
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Lymphoma ,Cancer ,Clinical Research ,Hematology ,Rare Diseases ,Aetiology ,2.1 Biological and endogenous factors ,Antigens ,CD ,Antigens ,CD1 ,Dendritic Cells ,Diagnosis ,Differential ,Female ,Gene Rearrangement ,B-Lymphocyte ,Hematologic Neoplasms ,Histiocytosis ,Langerhans-Cell ,Humans ,Immunohistochemistry ,In Situ Hybridization ,Fluorescence ,Lectins ,C-Type ,Lymphoma ,B-Cell ,Male ,Mannose-Binding Lectins ,Polymerase Chain Reaction ,S100 Proteins ,histiocytes ,indeterminate ,dendritic ,Langerhans. ,Birbeck granules ,Langerin ,Clinical Sciences ,Pathology - Abstract
Indeterminate cell tumor (ICT) is a rare neoplastic dendritic cell disorder that has been poorly defined due to its rarity and poorly understood histogenesis and pathogenesis. It is characterized by a proliferation of dendritic cells, which mimic Langerhans cells immunophenotypically (positive for CD1a and S-100 protein), but lack Birbeck granules characteristic of Langerhans cells. The clinical, morphologic, immunophenotypic, and ultrastructural features of 5 ICT cases are reported in an attempt to further define ICT and to examine the postulated relationship between indeterminate cells and Langerhans cells. Four of 5 patients were females, and 4 of 5 were older than 68 years. Three of 5 patients had cutaneous lesions, whereas 2 presented with cervical lymph node involvement. Two patients had a possible association with lymphoma: first patient had a history of progressive follicular lymphoma that led to patient's demise and the second patient had unexplained systemic lymphadenopathy and died 1 week after the biopsy. All 5 ICT cases expressed CD1a and S-100 protein, but lacked Langerin expression and Birbeck granules ultrastructurally. Interestingly, a t(14;18) was detected by fluorescence in situ hybridization in the ICT cells of the patient with previous follicular lymphoma and a monoclonal kappa light chain gene rearrangement was detected by polymerase chain reaction in the patient with systemic lymphadenopathy. In both cases, there was no morphologic or immunophenotypic evidence of a concurrent B-cell lymphoma. In conclusion, ICT is a rare neoplasm that can occur de novo or in association with a B-cell lymphoma, possibly as a result of B-cell dedifferentiation caused by relatively unknown mechanisms. Finally, Langerin immunostaining may be used as a surrogate marker for the ultrastructural demonstration of Birbeck granules, the absence of which represents a strong diagnostic criterion for ICT.
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- 2008
4. Indeterminate cell tumor: a rare dendritic neoplasm.
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Rezk, Sherif A, Spagnolo, Dominic V, Brynes, Russell K, and Weiss, Lawrence M
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Dendritic Cells ,Humans ,Lymphoma ,B-Cell ,Hematologic Neoplasms ,Histiocytosis ,Langerhans-Cell ,S100 Proteins ,Lectins ,C-Type ,Mannose-Binding Lectins ,Antigens ,CD ,Antigens ,CD1 ,Diagnosis ,Differential ,Immunohistochemistry ,In Situ Hybridization ,Fluorescence ,Polymerase Chain Reaction ,Gene Rearrangement ,B-Lymphocyte ,Female ,Male ,histiocytes ,indeterminate ,dendritic ,Langerhans. ,Birbeck granules ,Langerin ,Pathology ,Clinical Sciences - Abstract
Indeterminate cell tumor (ICT) is a rare neoplastic dendritic cell disorder that has been poorly defined due to its rarity and poorly understood histogenesis and pathogenesis. It is characterized by a proliferation of dendritic cells, which mimic Langerhans cells immunophenotypically (positive for CD1a and S-100 protein), but lack Birbeck granules characteristic of Langerhans cells. The clinical, morphologic, immunophenotypic, and ultrastructural features of 5 ICT cases are reported in an attempt to further define ICT and to examine the postulated relationship between indeterminate cells and Langerhans cells. Four of 5 patients were females, and 4 of 5 were older than 68 years. Three of 5 patients had cutaneous lesions, whereas 2 presented with cervical lymph node involvement. Two patients had a possible association with lymphoma: first patient had a history of progressive follicular lymphoma that led to patient's demise and the second patient had unexplained systemic lymphadenopathy and died 1 week after the biopsy. All 5 ICT cases expressed CD1a and S-100 protein, but lacked Langerin expression and Birbeck granules ultrastructurally. Interestingly, a t(14;18) was detected by fluorescence in situ hybridization in the ICT cells of the patient with previous follicular lymphoma and a monoclonal kappa light chain gene rearrangement was detected by polymerase chain reaction in the patient with systemic lymphadenopathy. In both cases, there was no morphologic or immunophenotypic evidence of a concurrent B-cell lymphoma. In conclusion, ICT is a rare neoplasm that can occur de novo or in association with a B-cell lymphoma, possibly as a result of B-cell dedifferentiation caused by relatively unknown mechanisms. Finally, Langerin immunostaining may be used as a surrogate marker for the ultrastructural demonstration of Birbeck granules, the absence of which represents a strong diagnostic criterion for ICT.
- Published
- 2008
5. A Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a+) Mimicking Glioblastoma Multiforme in a Young Adult.
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Bärtschi, Patrick, Luna, Enrique, González-López, Pablo, Abarca, Javier, Herrero, Joaquín, Costa, Estela, Paya, Artemio, Sales, Juan, and Moreno, Pedro
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INSULAR cortex , *LANGERHANS-cell histiocytosis , *PITUITARY gland , *ERDHEIM-Chester disease , *PULMONARY nodules , *LANGERHANS cells - Abstract
Background Langerhans cell histiocytosis (LCH) is a multisystemic dendritic cell proliferation that is relatively uncommon in adults. Central nervous system LCH outside the pituitary gland is even more uncommon. Case Description We report the case of a 42-year-old man who had complained of right-side hemicranial pain and left arm minor paresis. The symptoms were due to a right insular lobe heterogeneous-enhancing lesion associated with extensive vasogenic edema. The first diagnostic impression suggested glioblastoma multiforme or localized metastasis. The thoracic, abdominal, pelvic computed tomography scan only detected small upper lung inactive nodules suggesting silent focal LCH. A very hard lesion was almost completely removed through a pterional craniotomy approach, with no fluorescence after aminolevulinic acid infusion. The intraoperative biopsy findings ruled out glioma but could not confirm lymphoma. The definitive cerebral biopsy findings showed lymphocytes and histiocytes (CD1a+, S-1001+), with a diagnosis of intracerebral parenchymal LCH. Fractioned radiotherapy resulted in clinical and radiological remission. Conclusions The present case is so rare it should not be used as a guide. We probably will never see a single intraparenchymal supratentorial central nervous system LCH lesion. However, we hope our report will help colleagues in the future with the thought process. Highlights • LCH is a rare heterogeneous disease characterized by an accumulation of dendritic cells in various organs. • CNS involvement that excludes the pituitary gland is extremely uncommon. • A rare intraparenchymal radiological presentation can be misinterpreted as high-grade glioma. • A multispecialty approach should be mandatory when CNS LGH is suspected. [ABSTRACT FROM AUTHOR]
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- 2019
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6. The Essential Role of anxA2 in Langerhans Cell Birbeck Granules Formation
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Shantae M. Thornton, Varsha D. Samararatne, Joseph G. Skeate, Christopher Buser, Kim P. Lühen, Julia R. Taylor, Diane M. Da Silva, and W. Martin Kast
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anxA2 ,Birbeck granules ,Langerhans cell ,A2t ,Cytology ,QH573-671 - Abstract
Langerhans cells (LC) are the resident antigen presenting cells of the mucosal epithelium and play an essential role in initiating immune responses. LC are the only cells in the body to contain Birbeck granules (BG), which are unique cytoplasmic organelles comprised of c-type lectin langerin. Studies of BG have historically focused on morphological characterizations, but BG have also been implicated in viral antigen processing which suggests that they can serve a function in antiviral immunity. This study focused on investigating proteins that could be involved in BG formation to further characterize their structure using transmission electron microscopy (TEM). Here, we report a critical role for the protein annexin A2 (anxA2) in the proper formation of BG structures. When anxA2 expression is downregulated, langerin expression decreases, cytoplasmic BG are nearly ablated, and the presence of malformed BG-like structures increases. Furthermore, in the absence of anxA2, we found langerin was no longer localized to BG or BG-like structures. Taken together, these results indicate an essential role for anxA2 in facilitating the proper formation of BG.
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- 2020
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7. Telocyte-like cells containing Weibel–Palade bodies in rat lamina fusca.
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Petrea, C.E., Vrapciu, A.D., Rusu, M.C., and Mănoiu, V.S.
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ENDOTHELIAL cells ,STROMAL cells ,ERYTHROCYTES ,CELL membranes ,RATS ,TRANSMISSION electron microscopy - Abstract
Telocytes (TCs) are cells with long, thin and moniliform processes called telopodes. These cells have been found in numerous tissues, including the eye choroid and sclera. Lamina fusca (LF), an anatomical structure located at the sclera-choroid junction, has outer fibroblastic lamellae containing cells with long telopodes. The purpose of this study was to evaluate, via transmission electron microscopy, the LF for the presence of endothelial-specific ultrastructural features, such as Weibel–Palade bodies (WPBs), in the residing TCs. We found that the outer fibroblastic layer of LF lacked pigmented cells but contained numerous cells with telopodes. These cells had incomplete or absent basal laminae, were united by focal adhesions and close contacts, and displayed scarce caveolae and shedding vesicles. Within the stromal cells of LF, numerous WPBs in various stages of maturation and vesicular structures, as secretory pods that ensure the exocytosis of WPBs content, were observed. The WPBs content of the cells with telopodes in the LF could indicate either their involvement in vasculogenesis and/or lymphangiogenesis or that they are the P-selectin- and CD63-containing pools that play roles in scleral or choroidal inflammation. [ABSTRACT FROM AUTHOR]
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- 2018
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8. Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis.
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Ali-Ridha, Andre, Brownstein, Seymour, O'Connor, Michael, Gilberg, Steve, and Tang, Tina
- Abstract
Langerhans cell histiocytosis (LCH) is a rare multi-system disease. It presents infrequently as a childhood orbital tumor, and can mimic more common inflammatory orbital disease processes. We report the clinical, histopathological, and electron microscopic findings of orbital LCH in an 18-month-old child, along with a review of the recent literature regarding molecular pathogenetic analysis of LCH. The child presented with a two-week history of progressive left periorbital edema and redness. He was initially diagnosed and treated empirically for bacterial periorbital cellulitis, but subsequently underwent ophthalmological consultation after he failed to improve. Histopathological examination of an orbital biopsy specimen revealed numerous Langerhans-type cells, which stain positive for CD1A and CD207 (langerin). Electron microscopic examination demonstrated characteristic Birbeck granules within the Langerhans-type cells. Three year follow-up did not demonstrate recurrence or disease progression. [ABSTRACT FROM AUTHOR]
- Published
- 2018
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9. Indeterminate Dendritic Cell Tumor in Thoracic Spine: A Case Report.
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Tan, Sze Kiat, Chieng, Lee Onn, Madhavan, Karthik, Rosenberg, Andrew, and Cote, Ian
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DENDRITIC cells , *THORACIC vertebrae , *RETICULUM cell sarcoma , *CELL tumors , *LANGERHANS cells , *CHEST pain - Abstract
Background Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic disorder with poorly understood pathogenesis. Occasionally encountered by hematologists, unusual presentations of IDCT have not been reported in the spine literature. Methods We report a 51-year-old man who presented with a 3-month history of progressively worsening axial thoracic back pain radiating to his sides. Magnetic resonance imaging revealed a 3-cm enhancing mass at the T9 vertebral body with an exophytic component causing significant canal stenosis. Initial percutaneous biopsy revealed histiocytic sarcoma. Results The patient underwent exploratory thoracotomy and en bloc resection of the lesion with T8-10 fusion. Final pathology results revealed IDCT with fibrosis. IDCT immunostaining was partially positive for Langerhans cell marker (positive for S100 and CD1a, but lacked Birbeck granules and Langerin stain) and partially positive for blastic plasmacytoid dendritic cell neoplasm. Additionally, it was positive for CD45, CD68, and CD163. Lymphadenopathy was absent in this patient. Conclusions Although first reported in the 1980s, IDCT has been omitted from most classifications owing to its rarity. Hematologists have debated the cell of origin; it is believed to comprise pre–Langerhans cells, as Birbeck granules are acquired after migration to the epidermis. IDCT remains of indeterminate origin. We report the first case of spinal IDCT. Familiarity with the histologic features is warranted to ensure accurate diagnosis and appropriate treatment. [ABSTRACT FROM AUTHOR]
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- 2017
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10. Thymic Accessory Cells, Including Dendritic Type Antigen Presenting Cells, within the Mammalian Thymic Microenvironment
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Qayumi, Yasmin, Kaiser, Hans E., editor, Nasir, Aejaz, editor, Bodey, Bela, and Siegel, Stuart E.
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- 2004
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11. Identification of Langerhans-like cells in the immunocompetent tissues of channel catfish, Ictalurus punctatus.
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Kordon, Adef O., Scott, Matthew A., Ibrahim, Iman, Abdelhamed, Hossam, Ahmed, Hamada, Baumgartner, Wes, Karsi, Attila, and Pinchuk, Lesya M.
- Subjects
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LANGERHANS cells , *FISH immunology , *IMMUNOCOMPETENT cells , *DENDRITIC cells , *IMMUNOHISTOCHEMISTRY , *CHANNEL catfish - Abstract
Dendritic cells (DCs) are the most powerful antigen presenting cells (APCs) that have a critical role in bridging innate and adaptive immune responses in vertebrates. Dendritic cells have been characterized morphologically and functionally in the teleost fish models such as rainbow trout, salmonids, medaka, and zebrafish. The presence of DCs with remarkable similarities to human Langerhans cells (LCs) has been described in the spleen and anterior kidney of salmonids and rainbow trout. However, there is no evidence of the presence of DCs and their role in channel catfish immunity. In this study, we assessed DC-like cells in the immunocompetent tissues of channel catfish by immunohistochemistry (IHC), flow cytometry and transmission electron microscopy (TEM). We identified Langerin/CD207 + (L/CD207 + ) cells in the channel catfish anterior kidney, spleen and gill by IHC. Moreover, we described the cells that resembled mammal LC DCs containing Birbeck-like (BL) granules in channel catfish spleen, anterior and posterior kidneys and gill by TEM. Our data suggest that cells with DC-like morphology in the immune related organs of catfish may share morphological and functional properties with previously reported DCs in teleost fish and mammals. More detailed knowledge of the phenotype and the function of catfish DCs will not only help gain insight into the evolution of the vertebrate adaptive immune system but will also provide valuable information for development and optimization of immunotherapies and vaccination protocols for aquaculture use. [ABSTRACT FROM AUTHOR]
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- 2016
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12. Langerhans cell histiocytosis
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Carl E. Allen and Carlos Rodriguez-Galindo
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Proto-Oncogene Proteins B-raf ,Pathology ,medicine.medical_specialty ,MAP Kinase Signaling System ,Birbeck granules ,business.industry ,Immunology ,Cell Biology ,Hematology ,Disease ,Cystic Change ,medicine.disease ,Biochemistry ,Stain ,Histiocytosis, Langerhans-Cell ,Histiocytosis ,Langerhans cell histiocytosis ,Mutation ,medicine ,Animals ,Humans ,Lymph ,business ,Protein Kinase Inhibitors ,Histiocyte - Abstract
Langerhans cell histiocytosis (LCH) is caused by clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in lesions that leads to a spectrum of organ involvement and dysfunction. The pathogenic cells are defined by constitutive activation of the MAPK signaling pathway. Treatment of LCH is risk-adapted: patients with single lesions may respond well to local treatment, whereas patients with multisystem disease require systemic therapy. Although survival rates for patients without organ dysfunction is excellent, mortality rates for patients with organ dysfunction may reach 20%. Despite progress made in the treatment of LCH, disease reactivation rates remain above 30%, and standard second-line treatment is yet to be established. Treatment failure is associated with increased risks for death and long-term morbidity, including LCH-associated neurodegeneration. Early case series report promising clinical responses in patients with relapsed and refractory LCH treated with BRAF or MEK inhibitors, although potential for this strategy to achieve cure remains uncertain.
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- 2020
13. Langerhans cell histiocytosis: A great imitator
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Kayla St Claire, Marylee Braniecki, Michelle Bain, Ryan Bunney, Maria M. Tsoukas, and Kurt A. Ashack
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Pathology ,medicine.medical_specialty ,Langerin ,Birbeck granules ,Dermatology ,Skin Diseases ,Antigens, CD1 ,Diagnosis, Differential ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Langerhans cell histiocytosis ,Antigens, CD ,Seborrheic dermatitis ,Biopsy ,Eosinophilic ,medicine ,Humans ,Lectins, C-Type ,Skin ,030203 arthritis & rheumatology ,integumentary system ,biology ,medicine.diagnostic_test ,business.industry ,Biopsy, Needle ,S100 Proteins ,medicine.disease ,Histiocytosis, Langerhans-Cell ,Microscopy, Electron ,Histiocytosis ,Mannose-Binding Lectins ,biology.protein ,Differential diagnosis ,business ,Biomarkers - Abstract
Langerhans cell histiocytosis (LCH) is an uncommon but serious inflammatory neoplasia that affects many organs, including the skin. Though uncommon, it should remain high on a clinician's differential diagnosis in treatment-resistant cases of conditions, such as seborrheic dermatitis, diaper dermatitis, arthropod bites, and many more. A thorough history nd physical examination for each patient can aid in the diagnosis; however, if clinically suspicious for LCH, a punch biopsy should be performed. Histologic evaluation of LCH is often enough to differentiate it from the many clinical mimickers. Characteristic findings include a histiocytic infiltrate with "coffee bean"-cleaved nuclei, rounded shape, and eosinophilic cytoplasm. Immunohistochemical stains, including CD1a, S100, and CD207 (langerin) are often needed for a definitive diagnosis. Electron microscopy also demonstrates the ultrastructural presence of Birbeck granules, but this is no longer needed due to immunohistochemical staining. Treatment is often necessary for LCH, if systemic involvement exists.
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- 2020
14. Langerhans cell histiocytosis: Diagnosis and Management
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Githa Rahmayunita, Tjut Nurul Alam Jacoeb, Hikari Ambara Sjakti, Inge Ade Krisanti, and Lusiana Lusiana
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Pathology ,medicine.medical_specialty ,Lung ,Birbeck granules ,business.industry ,lcsh:RL1-803 ,medicine.disease ,Stain ,Lesion ,On cells ,Histiocytosis ,medicine.anatomical_structure ,Langerhans cell histiocytosis ,children ,medicine ,langerhans cells ,lcsh:Dermatology ,dendritic cells ,medicine.symptom ,business ,histiocytosis ,Histiocyte - Abstract
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal clonal proliferation of Langerhans dendritic cells. The incidence of LCH is 1 to 3 cases per 1 million children worldwide, and occurs most frequently in children of 1–4 years of age. The etiopathogenesis of LCH, whether it is neoplastic or reactive, is still controversial. Langerhans cell histiocytosis has a wide spectrum of clinical features, and dermatological abnormalities usually occur early. The most frequent lesions are elevated yellowish-red translucent papules about 1–2 mm in diameter and generally located in seborrheic areas. The most recent classification (the Histiocyte Society study 2017) categorized LCH into four groups; a single system with involvement of unifocal or multifocal organs, lung LCH, and multi-system LCH with either low- or high-risk multiorgan involvement. The definitive diagnosis of LCH are typical morphology along with Birbeck granules and/or positive results on CD1a antigen stain on cells found on lesions. Treatment of LCH is multimodal, determined based on age, extent of lesion, organ involvement, and organ location. Keywords: histiocytosis, Langerhans cells, dendritic cells, children
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- 2019
15. Solitary Extragnathic Langerhans Cell Histiocytosis – A Rare Case
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Vankayala Yashoda, Eppalapally Sharath Kumar Reddy, Sangala Naga Bhavani, Krishna A, and Mane Srinivas Muni Sekhar
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birbeck granules ,eosinophilic granuloma ,histiocytes ,langerhans cells ,ulcer ,Medicine - Abstract
Langerhans cell histiocytosis (LCH), mainly affects the skull, vertebrae, ribs and mandible in children and the long bones of adults. Symptoms range from none to pain, swelling and tenderness over the site of the lesion. This disease presents oral manifestations which can sometimes be the first expression of the condition. It occurs in three forms namely eosinophilic granuloma in which isolated or multiple bones are involved, But has a good prognosis whereas other variants Hand-Shuller-Christian disease (chronic dessiminated variant) and Letterer-Siwe disease (acute dessiminated form) have poor prognosis. Occasionally only soft tissues are affected without bony involvement. Males are more commonly affected than females. This article describes a rare variant of eosinophilic granuloma of labial mucosa without bony involvement.
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- 2015
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16. Langerhans Cell Histiocytosis Co-existing with Papillary Carcinoma of Thyroid
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Jaimanti Bakshi and Atul Kumar Goyal
- Subjects
Pathology ,medicine.medical_specialty ,endocrine system diseases ,Birbeck granules ,business.industry ,Thyroid ,medicine.disease ,Papillary thyroid cancer ,Vinblastine ,Thyroid carcinoma ,medicine.anatomical_structure ,Langerhans cell histiocytosis ,medicine ,business ,Infiltration (medical) ,Etoposide ,medicine.drug - Abstract
Langerhans cell histiocytosis (LCH) is a rare condition with unknown causes and is characterized by the infiltration of significant numbers of Langerhans cells in the affected tissues leading to formation of granulomas. In this chapter, we have discussed the case of a 31-year-old man with a swollen, diffusely firm, non-tender, non-mobile thyroid swelling presented with Langerhans cell histiocytosis associated with papillary thyroid carcinoma (PTC). The thyroid was shown to be involved by langerhans cell histiocytosis, confirmed with fine needle aspiration cytology. The patient was examined, and no signs of structural intervention were discovered. After ten cycles of vinblastine and etoposide chemotherapy, there was no reduction in the extent of the swelling observed.
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- 2021
17. Morphological Alterations and Increased S100B Expression in Epidermal Langerhans Cells Detected in Skin from Patients with Progressive Vitiligo
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Ichiro Katayama, Lanting Teng, Huimin Zhang, Fei Yang, and Lingli Yang
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0301 basic medicine ,vitiligo ,Pathology ,medicine.medical_specialty ,Langerin ,Birbeck granules ,Leukoderma ,Science ,Immunocytochemistry ,chemical and pharmacologic phenomena ,Vitiligo ,Birbeck granule ,S100B ,Article ,General Biochemistry, Genetics and Molecular Biology ,Pathogenesis ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,medicine ,leukoderma ,Langerhans cells ,skin and connective tissue diseases ,Ecology, Evolution, Behavior and Systematics ,biology ,Epidermis (botany) ,integumentary system ,Paleontology ,hemic and immune systems ,medicine.disease ,030104 developmental biology ,Space and Planetary Science ,biology.protein ,Ultrastructure - Abstract
The role of Langerhans cells (LCs) in vitiligo pathogenesis remains unclear, with published studies reporting contradictory results regarding the quantity of LCs and no data on the features of LCs in vitiligo. Here, we aimed to analyze the presence, density, and morphological features of LCs in the epidermis of patients with vitiligo. Skin biopsies were stained for LCs using anti-CD1a/anti-langerin antibodies and analyzed by immunocytochemistry with light and electron microscopy. Compared with healthy controls, we detected significantly increased numbers of epidermal LCs in lesional skin from vitiligo in the progressive state. These LCs exhibited striking morphological alterations, including an elevated number of dendrites, with increased length and more branches than dendrites from controls. Ultrastructure examination via immuno-electron microscopy revealed markedly reduced Birbeck granules (BGs) and shorter BG rods in LCs from progressive vitiligo, with higher expression of langerin. Additionally, expression of S100B, the activity biomarker of vitiligo, was increased in these LCs. This work provides new insight on the cellular composition of LCs in vitiliginous skin, revealing altered morphology and increased LC numbers, with elevated S100B expression. Our data suggest LCs might play a critical role in vitiligo pathogenesis and thus may represent a novel therapeutic target for this disease.
- Published
- 2021
18. Morphological characteristic and functional dependencies of dendritic cell in developing rabbit lung during fetal and neonatal life
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Doaa M. Mokhtar and Marwa M. Hussein
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Receptor, ErbB-2 ,Birbeck granules ,T-Lymphocytes ,Endocytic cycle ,Cell ,Antigen presentation ,chemical and pharmacologic phenomena ,CD8-Positive T-Lymphocytes ,Biology ,Endocytosis ,03 medical and health sciences ,Fetus ,0302 clinical medicine ,medicine ,Animals ,Lymphocytes ,Telocytes ,Lung ,Molecular Biology ,030304 developmental biology ,Antigen Presentation ,0303 health sciences ,Stem Cells ,Cell Differentiation ,hemic and immune systems ,Dendritic Cells ,Cell Biology ,Dendritic cell ,Immunohistochemistry ,Cell biology ,medicine.anatomical_structure ,Animals, Newborn ,Rabbits ,030217 neurology & neurosurgery ,CD8 ,Developmental Biology - Abstract
Recently, pulmonary DC deserved the attention of researchers and clinicians as it was implicated in many diseases afflicting human lungs. However, there are no available data about the morphological or functional features of pulmonary dendritic cells in fetal or early neonatal life. The present study aimed to demonstrate the morphological development of DCs using light-, electron-microscopy, and immunohistochemistry. DCs showed strong immunoreactivity for both CD8 and CD56. Moreover, DCs strongly expressed CD34, VEGF, NSE, and connexin-43 within the developing pulmonary tissue. By SEM, DCs were polyhedral in shape with short cell processes in fetal life. By the advancement of the age, DCs became more numerous and exhibited rounded to oval cell bodies with many fine dendrites. TEM revealed that at early fetal life, DCs were characterized by their heterochromatic indented nuclei, few cell processes and few organelles. With the advancement of age, DCs showed dendrite-like processes and displayed signs of high endocytic activities with releasing of secretory materials. At late fetal life, DCs showed an obvious increase in the nuclear/cytoplasmic ratio and they exhibited a unique connection with type II pneumocytes and pulmonary endothelium by gap junction. In the early neonate, the DCs cells were seen in association with T-lymphocytes, neutrophils, telocytes (TCs), and air-blood barrier. They possessed many fine dendrites, the characteristic Birbeck granules and many vesicles. DCs may contribute to apoptosis, endocytosis, and angiogenesis. The difference in the maturation status may reflect different roles for DCs in the lung. The immature DCs may have an antigen-uptake role through endocytosis, while mature DCs may involve in antigen presentation to T-cells.
- Published
- 2019
19. Post‐scabietic nodules: Mimicker of infantile indeterminate cell histiocytosis and potential diagnostic pitfall
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Eric Y. Loo, Robert E. LeBlanc, Julianne A. Mann, and Kristen N Ruby
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Male ,Pathology ,medicine.medical_specialty ,Histology ,Birbeck granules ,Context (language use) ,Dermatology ,Pathology and Forensic Medicine ,Scabies ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Humans ,Medicine ,Indeterminate Cell Histiocytosis ,business.industry ,Indeterminate Dendritic Cell Tumor ,Infant ,medicine.disease ,Histiocytosis, Langerhans-Cell ,Histiocytosis ,030220 oncology & carcinogenesis ,Etiology ,Indeterminate ,business ,Rare disease - Abstract
Indeterminate cell histiocytosis (ICH) is an extremely rare disease and little is known about its etiology. Patients usually present with nodular, dermal proliferations of indeterminate cells, which characteristically resemble Langerhans cells but lack Birbeck granules. The clinical course is highly variable, ranging from spontaneous regression to rapid progression with reports of extracutaneous involvement, subsequent acute myeloid leukemias, and associated B-cell lymphomas. Rare cases of ICH-like reactions have been reported in the setting of scabies infestations as well as in patients who had been bitten by ticks and mosquitos. We present a successfully treated case of indeterminate cell-rich post scabietic nodules in an otherwise healthy 8-month-old boy and review the literature on similar cases. Clinical context is essential for correct interpretation of these indolent ICH-mimicking lesions, and to avert unnecessary patient anxiety and aggressive management.
- Published
- 2019
20. A Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a+) Mimicking Glioblastoma Multiforme in a Young Adult
- Author
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Pablo González-López, Joaquín Herrero, Javier Abarca, Juan Sales, Pedro Moreno, Artemio Paya, Patrick Bärtschi, Enrique Luna, and Estela Costa
- Subjects
Pathology ,medicine.medical_specialty ,Langerhans cell ,Birbeck granules ,business.industry ,medicine.disease ,Lesion ,Dendritic cell proliferation ,03 medical and health sciences ,Histiocytosis ,0302 clinical medicine ,medicine.anatomical_structure ,Langerhans cell histiocytosis ,030220 oncology & carcinogenesis ,Glioma ,medicine ,Surgery ,Neurology (clinical) ,medicine.symptom ,business ,030217 neurology & neurosurgery ,Histiocyte - Abstract
Background Langerhans cell histiocytosis (LCH) is a multisystemic dendritic cell proliferation that is relatively uncommon in adults. Central nervous system LCH outside the pituitary gland is even more uncommon. Case Description We report the case of a 42-year-old man who had complained of right-side hemicranial pain and left arm minor paresis. The symptoms were due to a right insular lobe heterogeneous-enhancing lesion associated with extensive vasogenic edema. The first diagnostic impression suggested glioblastoma multiforme or localized metastasis. The thoracic, abdominal, pelvic computed tomography scan only detected small upper lung inactive nodules suggesting silent focal LCH. A very hard lesion was almost completely removed through a pterional craniotomy approach, with no fluorescence after aminolevulinic acid infusion. The intraoperative biopsy findings ruled out glioma but could not confirm lymphoma. The definitive cerebral biopsy findings showed lymphocytes and histiocytes (CD1a+, S-1001+), with a diagnosis of intracerebral parenchymal LCH. Fractioned radiotherapy resulted in clinical and radiological remission. Conclusions The present case is so rare it should not be used as a guide. We probably will never see a single intraparenchymal supratentorial central nervous system LCH lesion. However, we hope our report will help colleagues in the future with the thought process.
- Published
- 2019
21. Molecular Recognition in C-Type Lectins: The Cases of DC- SIGN, Langerin, MGL, and L-Sectin
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Química orgánica II, Kimika organikoa II, Valverde, Pablo, Martínez, J. Daniel, Cañada, F. Javier, Ardá, Ana, Jiménez Barbero, Jesús, Química orgánica II, Kimika organikoa II, Valverde, Pablo, Martínez, J. Daniel, Cañada, F. Javier, Ardá, Ana, and Jiménez Barbero, Jesús
- Abstract
Carbohydrates play a pivotal role in intercellular communication processes. In particular, glycan antigens are key for sustaining homeostasis, helping leukocytes to distinguish damaged tissues and invading pathogens from healthy tissues. From a structural perspective, this cross-talk is fairly complex, and multiple membrane proteins guide these recognition processes, including lectins and Toll-like receptors. Since the beginning of this century, lectins have become potential targets for therapeutics for controlling and/or avoiding the progression of pathologies derived from an incorrect immune outcome, including infectious processes, cancer, or autoimmune diseases. Therefore, a detailed knowledge of these receptors is mandatory for the development of specific treatments. In this review, we summarize the current knowledge about four key C-type lectins whose importance has been steadily growing in recent years, focusing in particular on how glycan recognition takes place at the molecular level, but also looking at recent progresses in the quest for therapeutics.
- Published
- 2020
22. Caveolin-1 mediated uptake via langerin restricts HIV-1 infection in human Langerhans cells.
- Author
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van den Berg, Linda M., Ribeiro, Carla M. S., Zijlstra-Willems, Esther M., de Witte, Lot, Fluitsma, Donna, Tigchelaar, Wikky, Everts, Vincent, and Geijtenbeek, Teunis B. H.
- Subjects
- *
LANGERHANS cells , *CAVEOLINS , *HIV prevention , *SEXUALLY transmitted diseases , *ENDOCYTOSIS - Abstract
Background Human Langerhans cells (LCs) reside in foreskin and vaginal mucosa and are the first immune cells to interact with HIV-1 during sexual transmission. LCs capture HIV-1 through the C-type lectin receptor langerin, which routes the virus into Birbeck granules (BGs), thereby preventing HIV-1 infection. BGs are langerin-positive organelles exclusively present in LCs, however, their origin and function are unknown. Results Here, we not only show that langerin and caveolin-1 co-localize at the cell membrane and in vesicles but also that BGs are langerin/caveolin-1-positive vesicles that are linked to the lysosomal degradation pathway in LCs. Moreover, inhibition of caveolar endocytosis in primary LCs abrogated HIV-1 sequestering into langerin+ caveolar structures. Notably, both inhibition of caveolar uptake and silencing of caveolar structure protein caveolin-1 resulted in increased HIV-1 integration and subsequent infection. In contrast, inhibition of clathrinmediated endocytosis did not affect HIV-1 integration, even though HIV-1 uptake was decreased, suggesting that clathrin-mediated endocytosis is not involved in HIV-1 restriction in LCs. Conclusions Thus, our data strongly indicate that BGs belong to the caveolar endocytosis pathway and that caveolin-1 mediated HIV-1 uptake is an intrinsic restriction mechanism present in human LCs that prevents HIV-1 infection. Harnessing this particular internalization pathway has the potential to facilitate strategies to combat HIV-1 transmission. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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23. Congenital 'self-healing' Langerhans cell histiocytosis ( Hashimoto- Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course.
- Author
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Mandel, Victor Desmond, Ferrari, Chiara, Cesinaro, Anna Maria, Pellacani, Giovanni, and Del Forno, Corrado
- Abstract
Congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease is a rare condition present at birth or in the neonatal period characterized by small reddish-brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long-term follow-up is mandatory in order to reveal possible systemic involvement. We describe two cases of congenital self-healing Langerhans cell histiocytosis with widespread and very similar cutaneous manifestations but different clinical course. The first patient had multisystemic disease (with lymph nodes, bones, liver and lungs affected) that required systemic therapy. The second patient had cutaneous and bony lesions that resolved spontaneously. We think that the adjective 'self-healing' is misleading and should be abandoned. We stress the importance of a complete systemic evaluation and the necessity of a long-term follow-up. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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24. Melatonin administration provokes the activity of dendritic reticular cells in the seminal vesicle of Soay ram during the non-breeding season
- Author
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Ahmed Hassan, Manal T Hussein, and Hanan H. Abd-Elhafeez
- Subjects
Male ,Cell biology ,Birbeck granules ,Molecular biology ,CD3 ,Science ,T-Lymphocytes ,Immunology ,chemical and pharmacologic phenomena ,Biology ,Article ,Melatonin ,symbols.namesake ,Seminal vesicle ,Antigen ,Reticular cell ,medicine ,Animals ,Lymphocytes ,Telocytes ,Multidisciplinary ,Innate immune system ,Sheep ,Macrophages ,Seminal Vesicles ,hemic and immune systems ,Dendritic Cells ,Golgi apparatus ,medicine.anatomical_structure ,biology.protein ,symbols ,Medicine ,Dendritic Cells, Follicular ,medicine.drug - Abstract
Dendritic cells (DCs) are innate immune cells which engulf, process and present antigens to the naïve T-lymphocyte cells. However, little is known about the effect of melatonin on the DCs. The present study aimed to investigate the morphology and distribution of the DCs by transmission electron microscopy and Immunohistochemistry after melatonin administration. A total of 8 out of 15 adult ram was randomly selected to receive the melatonin implant and the remaining 7 animals received melatonin free implants. DCs showed positive immunoreactivity for CD117, S-100 protein and CD34. There is an obvious increase in the number of the positive immunoreactive cells to CD3, estrogen receptor alpha and progesterone in the treated groups. The expression of CD56 and MHCII in the DCs was abundant in the treated groups. The ultrastructure study revealed that melatonin exerts a stimulatory effect on the DCs which was associated with increment in the secretory activity of DCs. The secretory activity demarcated by an obvious increase in the number of mitochondria, cisternae of rER and a well-developed Golgi apparatus. The endosomal- lysosomal system was more developed in the treated groups. A rod-shaped Birbeck granule was demonstrated in the cytoplasm of the melatonin treated group. DCs were observed in a close contact to telocytes, T-Lymphocytes, nerve fibers and blood vessels. Taken together, melatonin administration elicits a stimulatory action on the DCs and macrophages through increasing the size, the number and the endosomal compartments which may correlate to increased immunity.
- Published
- 2021
25. Indeterminate dendritic cell tumor in the pancreas
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Yassir El Baz, Michael Schmid, Thomas Steffen, Rosita Sortino, Antonia Loosen, Ignazio Tarantino, Fariba Abbassi, and Bruno M. Schmied
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medicine.medical_specialty ,Pathology ,AcademicSubjects/MED00910 ,Langerin ,Birbeck granules ,pancreatic cancer ,Case Report ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,jscrep/080 ,Pancreatic cancer ,Internal medicine ,medicine ,histiocytic pancreatic tumor ,indeterminate dendritic cell tumor ,Hematology ,biology ,business.industry ,Indeterminate Dendritic Cell Tumor ,medicine.disease ,dendritic cell tumor ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,biology.protein ,Immunohistochemistry ,Surgery ,Pancreas ,business ,Indeterminate - Abstract
Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic neoplastic disorder with proliferation of indeterminate dendritic cells. In the vast majority of cases, IDCTs are restricted to the skin or lymph nodes. To our knowledge, we report the first case of IDCT in the pancreas. Due to the rarity of extracutaneous IDCT, guidelines or treatment recommendations addressing their management are missing. We performed a review of literature to compare our experience to the management of other extracutaneous IDCT. Histopathological examination confirms the diagnosis of IDCT in electron microscopy and/or immunohistochemistry. Specific features are the lack of Birbeck granules and the nonreaction to Langerin antibodies. Concerning the aftercare of extracutaneous IDCT, we recommend a dermatological examination to rule out an additional cutaneous manifestation as well as annual blood examinations due to the association between IDCT and hematologic malignancies.
- Published
- 2020
26. BMP7 aberrantly induced in the psoriatic epidermis instructs inflammation-associated Langerhans cells
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Christina Passegger, Gerhard G. Thallinger, Theresa Benezeder, Melanie Spies, René Köffel, Clemens Painsi, Elisabeth Glitzner-Zeis, Julia Feichtinger, Tommaso Sconocchia, Julia Tittes, Anna Redl, Carmen Tam-Amersdorfer, Corinna Krump, Herbert Strobl, Georg Stary, Mathias Hochgerner, Peter Wolf, Maria Sibilia, and Izabela Borek
- Subjects
Adult ,CD4-Positive T-Lymphocytes ,Keratinocytes ,Male ,Langerhans cell ,Birbeck granules ,medicine.medical_treatment ,Bone Morphogenetic Protein 7 ,Immunology ,Mice, Transgenic ,610 Medicine & health ,Bone morphogenetic protein ,Lymphocyte Activation ,Transforming Growth Factor beta1 ,03 medical and health sciences ,Mice ,Young Adult ,0302 clinical medicine ,Psoriasis ,medicine ,Immunology and Allergy ,Animals ,Humans ,Cells, Cultured ,030304 developmental biology ,Aged ,Inflammation ,0303 health sciences ,Epidermis (botany) ,integumentary system ,business.industry ,Dendritic cell ,Middle Aged ,medicine.disease ,Cytokine ,medicine.anatomical_structure ,Gene Expression Regulation ,Langerhans Cells ,Bone Morphogenetic Proteins ,Cancer research ,Cytokines ,Female ,Epidermis ,Keratinocyte ,business ,030215 immunology ,Signal Transduction - Abstract
Background Epidermal hyperplasia represents a morphologic hallmark of psoriatic skin lesions. Langerhans cells (LCs) in the psoriatic epidermis engage with keratinocytes (KCs) in tight physical interactions; moreover, they induce T-cell-mediated immune responses critical to psoriasis. Objective This study sought to improve the understanding of epidermal factors in psoriasis pathogenesis. Methods BMP7-LCs versus TGF-β1-LCs were phenotypically characterized and their functional properties were analyzed using flow cytometry, cell kinetic studies, co-culture with CD4 T cells, and cytokine measurements. Furthermore, immunohistology of healthy and psoriatic skin was performed. Additionally, in vivo experiments with Junf/fJunBf/f K5cre-ERT mice were carried out to assess the role of bone morphogenetic protein (BMP) signaling in psoriatic skin inflammation. Results This study identified a KC-derived signal (ie, BMP signaling) to promote epidermal changes in psoriasis. Whereas BMP7 is strictly confined to the basal KC layer in the healthy skin, it is expressed at high levels throughout the lesional psoriatic epidermis. BMP7 instructs precursor cells to differentiate into LCs that phenotypically resemble psoriatic LCs. These BMP7-LCs exhibit proliferative activity and increased sensitivity to bacterial stimulation. Moreover, aberrant high BMP signaling in the lesional epidermis is mediated by a KC intrinsic mechanism, as suggested from murine data and clinical outcome after topical antipsoriatic treatment in human patients. Conclusions These data indicate that available TGF-β family members within the lesional psoriatic epidermis preferentially signal through the canonical BMP signaling cascade to instruct inflammatory-type LCs and to promote psoriatic epidermal changes. Targeting BMP signaling might allow to therapeutically interfere with cutaneous psoriatic manifestations.
- Published
- 2020
27. LCH with Multisystemic Involvement-A Case Report
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Shreya Sehgal, Lokesh Singh, Muniraju Maralakunte, and Uma Debi
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Pathology ,medicine.medical_specialty ,business.industry ,Birbeck granules ,Adult population ,General Medicine ,Disease ,medicine.disease ,Stain ,Langerhans cell histiocytosis ,Antigen ,Male patient ,Medicine ,business ,Paediatric population - Abstract
Langerhans cell histiocytosis (LCH) constitutes a rare group of disorder derived from the macrophages and dendritic cells. The cells in LCH show close similarity to the langerhans cells found in the skin and mucosa and stain positive for CD1a, S100, and CD207 antigens. These cells display the same intracytoplasmic organelles on electron microscopy as seen in Langerhans cells i.e. Birbeck granules. LCH is ten times more prevalent in the paediatric age group as compared to adult population. Females are affected more commonly as compared to the males. LCH can have single- or multi-system types of involvement. Unifocal or multifocal pattern is seen in singlesystem type of disease. The multisystem pattern is often seen in the paediatric population while unisystem pattern is seen in adults. We present case report of a 35 yrs old male patient with multisystemic involvement.
- Published
- 2020
28. Indeterminate Dendritic Cell Tumor as Cutaneous Involvement of Chronic Myelomonocytic Leukemia Successfully Treated With Phototherapy
- Author
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Mario Medina-Miguelañez, Marta Izquierdo, David Moyano-Bueno, Teresa Martínez, Angel Santos-Briz, María S. García-Sánchez, and Alex Viñolas-Cuadros
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Skin Neoplasms ,Birbeck granules ,Chronic myelomonocytic leukemia ,Dermatology ,Pathology and Forensic Medicine ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,hemic and lymphatic diseases ,medicine ,Humans ,Indeterminate Cell Histiocytosis ,Chronic ,Aged, 80 and over ,business.industry ,Indeterminate Dendritic Cell Tumor ,Leukemia, Myelomonocytic, Chronic ,General Medicine ,Dendritic cell ,Phototherapy ,medicine.disease ,Myelomonocytic leukemia ,Cutaneous Involvement ,Indeterminate cell histiocytosis ,Langerhans Cells ,Next-generation sequencing ,Indeterminate dendritic cell tumor ,Indeterminate ,business ,Rare disease - Abstract
Indeterminate dendritic cell tumor (IDCT) is a rare disease composed of so-called indeterminate cells, a dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells, but lacking Birbeck granules. We report a case of cutaneous IDCT occurring in a patient with chronic myelomonocytic leukemia (CMML) successfully treated with UV-A phototherapy. Next-generation sequencing studies of the CMML demonstrated mutations in TET2, ASXL1, and ZRS2 genes, also detected in the IDCT, demonstrating a clonal relationship between both tumors and confirming IDCT as a specific subtype in the spectrum of CMML-related cutaneous lesions.
- Published
- 2020
29. 55-year-old man with ulcers in inguinal fold and intergluteal cleft found to have systemic Langerhans cell histiocytosis
- Author
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Euphemia W. Mu, Jo-Ann Latkowski, Zhiheng Pei, Nicholas D. Cassai, Hao Feng, Shane A Meehan, and Nigar Anjuman Khurram
- Subjects
medicine.medical_specialty ,business.industry ,Birbeck granules ,Intergluteal cleft ,Inguinal fold ,Dermatology ,medicine.disease ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Langerhans cell histiocytosis ,030220 oncology & carcinogenesis ,Medicine ,business - Published
- 2018
30. HIV-1 replicates and persists in vaginal epithelial dendritic cells
- Author
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Jean-Robert Larrieux, Manish Sagar, Andrew J. Henderson, Luis M. Agosto, Suryaram Gummuluru, Alex Olson, Hisashi Akiyama, Victor Pena-Cruz, and Yvetane Moreau
- Subjects
0301 basic medicine ,integumentary system ,biology ,Langerin ,Chemokine receptor CCR5 ,Birbeck granules ,General Medicine ,CXCR4 ,Virus ,Epithelium ,3. Good health ,Cell biology ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,medicine.anatomical_structure ,Viral life cycle ,030220 oncology & carcinogenesis ,medicine ,biology.protein ,Receptor - Abstract
HIV-1 acquisition occurs most commonly after sexual contact. To establish infection, HIV-1 must infect cells that support high-level replication, namely CD4+ T cells, which are absent from the outermost genital epithelium. Dendritic cells (DCs), present in mucosal epithelia, potentially facilitate HIV-1 acquisition. We show that vaginal epithelial DCs, termed CD1a+ VEDCs, are unlike other blood- and tissue-derived DCs because they express langerin but not DC-SIGN, and unlike skin-based langerin+ DC subset Langerhans cells (LCs), they do not harbor Birbeck granules. Individuals primarily acquire HIV-1 that utilizes the CCR5 receptor (termed either R5 or R5X4) during heterosexual transmission, and the mechanism for the block against variants that only use the CXCR4 receptor (classified as X4) remains unclear. We show that X4 as compared with R5 HIV-1 shows limited to no replication in CD1a+ VEDCs. This differential replication occurs after fusion, suggesting that receptor usage influences postentry steps in the virus life cycle. Furthermore, CD1a+ VEDCs isolated from HIV-1-infected virologically suppressed women harbor HIV-1 DNA. Thus, CD1a+ VEDCs are potentially infected early during heterosexual transmission and also retain virus during treatment. Understanding the interplay between HIV-1 and CD1a+ VEDCs is important for future prevention and cure strategies.
- Published
- 2018
31. Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis
- Author
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Seymour Brownstein, Andre Ali-Ridha, Tina Tang, Michael O'Connor, and Steve Gilberg
- Subjects
medicine.medical_specialty ,Langerin ,Birbeck granules ,Periorbital Edema ,Case Report ,Stain ,03 medical and health sciences ,0302 clinical medicine ,lcsh:Ophthalmology ,Langerhans cell histiocytosis ,030225 pediatrics ,Biopsy ,Medicine ,integumentary system ,medicine.diagnostic_test ,biology ,business.industry ,Periorbital cellulitis ,Disease progression ,medicine.disease ,Dermatology ,Ophthalmology ,lcsh:RE1-994 ,030221 ophthalmology & optometry ,biology.protein ,business - Abstract
Langerhans cell histiocytosis (LCH) is a rare multi-system disease. It presents infrequently as a childhood orbital tumor, and can mimic more common inflammatory orbital disease processes. We report the clinical, histopathological, and electron microscopic findings of orbital LCH in an 18-month-old child, along with a review of the recent literature regarding molecular pathogenetic analysis of LCH. The child presented with a two-week history of progressive left periorbital edema and redness. He was initially diagnosed and treated empirically for bacterial periorbital cellulitis, but subsequently underwent ophthalmological consultation after he failed to improve. Histopathological examination of an orbital biopsy specimen revealed numerous Langerhans-type cells, which stain positive for CD1A and CD207 (langerin). Electron microscopic examination demonstrated characteristic Birbeck granules within the Langerhans-type cells. Three year follow-up did not demonstrate recurrence or disease progression. Keywords: Langerhans cell histiocytosis, Langerin, Periorbital cellulitis, Birbeck granules, LCH-III protocol
- Published
- 2018
32. Indeterminate Dendritic Cell Tumor in Thoracic Spine: A Case Report
- Author
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Karthik Madhavan, Lee Onn Chieng, Sze Kiat Tan, Andrew E. Rosenberg, and Ian Cote
- Subjects
Male ,0301 basic medicine ,Pathology ,medicine.medical_specialty ,Langerhans cell ,Langerin ,Birbeck granules ,Histiocytic sarcoma ,Thoracic Vertebrae ,Diagnosis, Differential ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Spinal Neoplasms ,biology ,medicine.diagnostic_test ,CD68 ,business.industry ,Indeterminate Dendritic Cell Tumor ,Magnetic resonance imaging ,Dendritic Cells ,Middle Aged ,medicine.disease ,Stenosis ,Spinal Fusion ,030104 developmental biology ,medicine.anatomical_structure ,Hematologic Neoplasms ,030220 oncology & carcinogenesis ,biology.protein ,Surgery ,Neurology (clinical) ,business ,Diskectomy - Abstract
Background Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic disorder with poorly understood pathogenesis. Occasionally encountered by hematologists, unusual presentations of IDCT have not been reported in the spine literature. Methods We report a 51-year-old man who presented with a 3-month history of progressively worsening axial thoracic back pain radiating to his sides. Magnetic resonance imaging revealed a 3-cm enhancing mass at the T9 vertebral body with an exophytic component causing significant canal stenosis. Initial percutaneous biopsy revealed histiocytic sarcoma. Results The patient underwent exploratory thoracotomy and en bloc resection of the lesion with T8-10 fusion. Final pathology results revealed IDCT with fibrosis. IDCT immunostaining was partially positive for Langerhans cell marker (positive for S100 and CD1a, but lacked Birbeck granules and Langerin stain) and partially positive for blastic plasmacytoid dendritic cell neoplasm. Additionally, it was positive for CD45, CD68, and CD163. Lymphadenopathy was absent in this patient. Conclusions Although first reported in the 1980s, IDCT has been omitted from most classifications owing to its rarity. Hematologists have debated the cell of origin; it is believed to comprise pre–Langerhans cells, as Birbeck granules are acquired after migration to the epidermis. IDCT remains of indeterminate origin. We report the first case of spinal IDCT. Familiarity with the histologic features is warranted to ensure accurate diagnosis and appropriate treatment.
- Published
- 2017
33. Langerhans cell histiocytosis of the head and neck in the pediatric population in a tertiary center: Clinical presentation, classification and treatment.
- Author
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García Díaz, Ma Piedad, De Luca Sologaistoa, Armin, De Paula Vernetta, Carlos, Moreno, M Andres, Galeano Paniagua, Laura Andrea, Fernández, Manuel Mateos, and Carceller, Miguel Armengot
- Subjects
- *
LANGERHANS-cell histiocytosis , *CHILD patients , *SYMPTOMS , *EAR canal , *TEMPORAL bone - Abstract
Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology with a heterogeneous and unspecific clinical presentation. Any organ or system may be involved but the most frequent is the skeleton. The diagnostic gold standard is done through histopathology combined with immunohistochemistry in the correct clinical setting. Treatment is still controversial. The objective of this study is to present a series of pediatric patients in order to contribute to the diagnosis and treatment of LCH. A longitudinal, descriptive and retrospective study was done in 20 pediatric patients histologically diagnosed of LCH int the last 10 years, in a tertiary center. A review of the medical history was done and data collection included: epidemiological data, clinical manifestations and classification, lesion characteristics, treatment employed, follow-up and evolution. The mean age presentation was 6.5 years (2–12). The most frequent disease presentation was a palpable skull tumor followed by headache. Other otorhinolaryngologic presenting symptoms were bloody otorrhea and post auricular swelling. Single-systems LCH with skull tumors was the most frequent classification. Treatment options (surgery, chemotherapy and corticosteroid infiltration) were indicated according to disease classification. LCH should be suspected in pediatric patients with a palpable cranial vault tumor, as well as in patients with chronic otorrhea with granulation tissue at the external ear canal. Management of LCH fundamentally includes chemotherapy and surgery, or the combination of both. High-risk organs lesions, including temporal bone lesions, and multi-system LCH are predictors of recurrence and should have a long-term follow-up. [ABSTRACT FROM AUTHOR]
- Published
- 2022
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34. Langerhans cell sarcoma: a case report.
- Author
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Valentín‐Nogueras, Sheila M., Seijo‐Montes, Rachelle, Montalván‐Miró, Elena, and Sánchez, Jorge L.
- Subjects
- *
LANGERHANS cells , *MACROPHAGES , *DENDRITIC cells , *TUMORS , *IMMUNOHISTOCHEMISTRY - Abstract
Primary cutaneous neoplasms of histiocytes and dendritic cells are rare. Langerhans cells are a subset of antigen-presenting dendritic cells. Neoplasms of Langerhans cells are classified into cytologically benign Langerhans cell histiocytosis and cytologically malignant Langerhans cell sarcoma. Langerhans cell sarcoma is a rare entity characterized by multiorgan involvement and an aggressive clinical course. To date, only 30 cases of Langerhans cell sarcoma, including the present case, have been reported. We report a new case of Langerhans cell sarcoma that presented with multifocal cutaneous involvement. Diagnosis was done based on histopathological, immunohistochemical evaluation, as well as ultrastructural analysis identifying the presence of Birbeck granules. Our case represents a new case of this extremely rare, overtly aggressive neoplasm of Langerhans cells. Within 2 years of diagnosis, the patient developed metastatic disease and consequently died. Early recognition is important because of the tendency of Langerhans cell sarcoma to recur and metastasize. Therefore, ancillary techniques such as immunohistochemical and ultrastructural studies to confirm the diagnosis are very advantageous. [ABSTRACT FROM AUTHOR]
- Published
- 2013
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35. Educational Case: Langerhans cell histiocytosis.
- Author
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Light J, Retrouvey M, and Conran RM
- Abstract
Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
- Published
- 2022
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36. Indeterminate cell tumor of the spleen.
- Author
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Chen, Mingyi, Agrawal, Renuka, Nasseri-Nik, Niloofar, Sloman, Andrew, and Weiss, Lawrence M.
- Subjects
SPLEEN tumors ,HISTOPATHOLOGY ,IMMUNOHISTOCHEMISTRY ,CELL nuclei ,ELECTRON microscopy ,DENDRITIC cells - Abstract
Summary: Indeterminate cell tumor is an extremely rare neoplasm that mainly occurs in the skin. We report a case of indeterminate cell tumor arising from the spleen, a previously unreported site for indeterminate cell tumor. Histologically, the tumor showed nests, nodules, and sheets of large polygonal cells with mostly oval nuclei; open chromatin; variable nucleoli; and abundant, eosinophilic cytoplasm. Some cells possessed irregularly convoluted nuclei with nuclear grooves and granular cytoplasm, suggestive of Langerhans cells. Immunohistochemically, the tumor cells were diffusely positive for S-100 and CD1a and negative for Langerin. No Birbeck granules were found by electron microscopy. Clinical and radiologic examination showed no other organomegaly or lymphadenopathy. A diagnosis of primary indeterminate cell tumor of the spleen was rendered. To the best of our knowledge, this is the first indeterminate cell tumor reported in the spleen. Biologic insights into dendritic cells in the spleen and the pertinent literature on these entities are reviewed. [Copyright &y& Elsevier]
- Published
- 2012
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37. Ontogeny and disease responses of Langerhans-like cells in lymphoid tissues of salmonid fish.
- Author
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Lovy, Jan, Savidant, Gayle, and Wright, Glenda
- Subjects
- *
ATLANTIC salmon , *LANGERHANS cells , *ONTOGENY , *LYMPHOID tissue , *IMMUNE response , *BIOMARKERS , *IMMUNOHISTOCHEMISTRY , *FISH diseases - Abstract
The ontogeny and disease responses of Langerhans-like cells within lymphoid tissues of Atlantic salmon, Salmo salar, and rainbow trout, Oncorhynchus mykiss, were investigated. These cells were studied in situ with the use of two markers: the ultrastructural presence of Birbeck-like granules and immunohistochemistry with an antibody against human langerin/CD207 that cross-reacts with salmonid tissues. The appearance of Birbeck-like granules was observed in rainbow trout at 2 weeks post-hatch (PH) in the thymus and anterior kidney prior to the development of the spleen. Spleen first appeared at 3 weeks PH in both Atlantic salmon and rainbow trout, and Birbeck-like granules were observed within cells of the newly developed spleens. The cross-reactivity of langerin as seen by immunohistochemistry was not clearly observed in kidney and spleen until 9 weeks PH, when a strong cytoplasmic reaction was observed. To study langerin-positive cells in spleen and kidney during disease, microsporidial gill disease (MGD) in rainbow trout was used as a known disease model inducing a strong cell-mediated adaptive immune response. Langerin-positive cells in healthy fish were seen predominantly in the spleen, and only low numbers were present in the anterior kidney. During MGD, langerin-positive cell numbers were elevated in the anterior kidney and were significantly higher during 5, 6, and 10 weeks post-exposure (PE) compared with healthy control tissue. During MGD, the distribution of langerin-positive cells in the spleen and anterior kidney shifted from having significantly higher numbers of cells in the spleen than in the kidney in controls and at 1 and 4 weeks PE to having a similar distribution of the cells in the two organs at 2, 3, 5, and 6 weeks PE. By 10 weeks PE, significantly higher numbers of langerin-positive cells occurred in the anterior kidney compared with the spleen. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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38. Histiocytosis X: Characteristics, behavior, and treatments as illustrated in a case series.
- Author
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Kasper, Ekkehard M., Aguirre-Padilla, David H., Alter, Raanan Y., and Anderson, Matthew
- Abstract
Background: Langerhans cell histiocytosis (LCH) is a proliferative disorder predominantly found in children. It often presents with pain in calvarium or spine and may cause neuroendocrine symptoms. The gold standard for diagnosing LCH is the detection of Birbeck Granules by EM. Here, we describe two unique presentations of LCH and we review current treatment guidelines. Case Description: The first patient was a 23-year-old man who presented with progressive swelling and redness of the left eye. MRI revealed a left retrobulbar lesion extending into the middle cranial fossa with no signal abnormality in the brain parenchyma. The lesion was resected and pathological analysis revealed LCH. Bone scans were negative and the patient was discharged soon after. He later underwent fractionated radiotherapy (cumulative dose 26 Gy). Follow-up MRIs show no disease at 24 months post-op. The second patient was a 56-year-old man with left frontal skull pain for 5 months. Imaging showed a solitary osteolytic lesion extending into both dura and scalp with no signal abnormality of the parenchyma. Excisional biopsy revealed LCH. Surgery was well tolerated and follow-up imaging shows no recurrence at 24 months post-op. Conclusion: We demonstrate that LCH, though uncommon, must remain on the differential when osteolytic lesions present in the adult. Although LCH often has the clinical and radiographical presentation of an abscess, pathology analysis can successfully diagnose LCH based on markers and morphological characteristics. LCH has an excellent prognosis when treated aggressively with surgical resection and radiotherapy as both of our patients were and are now disease free at 2 year follow-up. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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39. Lessons to be learned — Langerhans' cell histiocytosis.
- Author
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Dewan, Madhu, Al-Ghamdi, Abdulmonem Almutawa, and Zahrani, Mubarak B.
- Abstract
Langerhans' cell histiocytosis (LCH) is a neoplastic disease due to uncontrolled proliferation of Langerhans' cells (LCs); damage to organs involved appears to be due to the various cytokines secreted. The true cause of LCH remains a mystery. LCH can present at any age; there is female predominance and the skin is often the first site to be involved. Scaly papules, vesicles, purpuric nodules, plaques and ulcers are the main diverse presentations typically found on the scalp, flexures (including the perineum), crease below the breast, palms, soles and nails. The patient described here is a 35-year-old Saudi Arabian woman with a vulval ulcer that had been misdiagnosed as an infective lesion 11 months before the correct diagnosis of LCH was made by means of biopsy examination. Diffuse infiltration of LCs with large pale reniform/convoluted nuclei and abundant amphophilic cytoplasm in the company of neutrophils, eosinophils, lymphocytes and plasma cells were observed on light microscopy. Immunohistochemical staining, indicating S100 protein positivity in LCs and the demonstration of Birbeck granules by means of electron microscopy, confirmed the diagnosis.The `skin-only' LCH may later progress to involve other organs; alternatively skin lesions may be part of the multisystem, disseminated disease. There is need for careful follow-up of all LCH patients, as the course of the disease cannot be reliably predicted from either the clinical presentation or histology of the lesion. In many cases the prognosis is good, there being limited involvement that does not progress. Surgery or local steroids might suffice as therapy. However, for severely symptomatic disease, in which there are complications associated with dysfunction (or failure) of vital organs such as the liver, lungs, and central nervous system, the use of a variety of immunosuppressive treatments — and the recently described 2-chloro-2-deoxyadenosine (2CdA) (cladribine) — have been suggested; however, the type and extent of disease that will respond to this therapy has not yet been clarified. Solid laboratory research and rigorous clinical trials are required for more effective and less toxic therapies for patients with progressive LCH. [ABSTRACT FROM PUBLISHER]
- Published
- 2008
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40. Cutaneous Indeterminate Cell Histiocytosis of Donor Origin After Allogeneic Hematopoietic Stem-Cell Transplantation
- Author
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Angel Santos-Briz, Lourdes Vázquez, María Dolores Ludeña, Alvaro de Dios, Concepción Román, and Rocío Corral
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Birbeck granules ,medicine.medical_treatment ,Dermatology ,Hematopoietic stem cell transplantation ,Skin Diseases ,Pathology and Forensic Medicine ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Indeterminate Cell Histiocytosis ,business.industry ,Myelodysplastic syndromes ,Hematopoietic Stem Cell Transplantation ,General Medicine ,Dendritic cell ,Middle Aged ,medicine.disease ,Transplantation ,Histiocytosis ,Haematopoiesis ,Myelodysplastic Syndromes ,030220 oncology & carcinogenesis ,business - Abstract
Allogeneic hematopoietic stem-cell transplantation and solid-organ transplantation are associated with an increased risk of secondary neoplasms. Indeterminate cell histiocytosis (ICH) is a rare disease composed of so-called indeterminate cells, an alleged cutaneous dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells but lacking Birbeck granules. We report a case of cutaneous ICH occurring after allogeneic hematopoietic stem-cell transplantation for a myelodysplastic syndrome in a 56-year-old man. Microsatellite analysis demonstrated that the neoplastic cells were derived from the donor's hematopoietic system. This case broadens the spectrum of complications after stem-cell transplantation and demonstrates that cutaneous ICH in the setting of myelodysplastic syndromes may have a nonrelated origin to dysplastic myeloid cells.
- Published
- 2017
41. LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS
- Author
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Anil K Jain, Ish Kumar Dhammi, Rehan Ul-Haq, Himanshu Bhayana, Rajesh Kumar Rajneesh, and Rohit Pandey
- Subjects
Pathology ,medicine.medical_specialty ,lcsh:Diseases of the musculoskeletal system ,Birbeck granules ,03 medical and health sciences ,0302 clinical medicine ,lcsh:Orthopedic surgery ,Langerhans cell histiocytosis ,Scapula ,030225 pediatrics ,Biopsy ,medicine ,Orthopedics and Sports Medicine ,Histiocyte ,Histiocytosis, Langerhans-cell ,medicine.diagnostic_test ,business.industry ,CD68 ,medicine.disease ,Immunohistochemistry ,lcsh:RD701-811 ,Histiocytosis ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Surgery ,Neurology (clinical) ,Bone marrow ,lcsh:RC925-935 ,business - Abstract
Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presenta -tion of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.Keywords: Histiocytosis, Langerhans-cell; Scapula; Immunohistochemistry.
- Published
- 2017
42. Langerhans cell histiocytosis – a challenge for the dental professional
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Arun Garg, Shweta Bansal, and Richa Khurana
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Pathology ,medicine.medical_specialty ,Langerhans cell ,Histiocytosis-X ,Birbeck granules ,lcsh:Surgery ,Case Report ,Physical examination ,Dermatology ,03 medical and health sciences ,0302 clinical medicine ,Langerhans cell histiocytosis ,medicine ,Stage (cooking) ,periodontitis ,Periodontitis ,medicine.diagnostic_test ,business.industry ,Soft tissue ,030206 dentistry ,lcsh:RD1-811 ,medicine.disease ,oral manifestations ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Surgery ,Lymph ,business - Abstract
Langerhans cell histiocytosis (LCH) is a group of rare disorders histologically characterized by the proliferation of LC, involving multiple organs and systems. Typically, there is bone involvement and, less frequently, lesions may be found in the lungs, liver, lymph nodes, skin, and mucosae. Oral soft tissue lesions without bone involvement are rare. Antigenic markers that react with CD1a glycoprotein, cytoplasmatic protein S100 detected by immunoperoxidase staining, and/or presence of Birbeck granules on electron microscopic examination are required for a definitive diagnosis of LCH. In this article, we report a case of LCH, which had presented with multiple oral lesions without any other systemic signs and symptoms. Management of such children with periodontal manifestations should include hematological and, if possible, immunological investigations at an early stage. Careful clinical examination, good diagnostic skill, and awareness of characteristic cytological features of LCH can lead to earlier diagnosis and treatment with minimal deformity.
- Published
- 2017
43. A human Langerhans cell-like cell line, ELD-1, promotes CD8 T cells to produce IFN-γ through CD70-dependent alternative pathway
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Iwamoto, Sanju, Ishida, Makoto, Tamaoki, Satoshi, Hagiwara, Tamio, Sueki, Hirohiko, and Miyazaki, Akira
- Subjects
- *
LANGERHANS cells , *LYMPHOID tissue , *ANTINEOPLASTIC agents , *ANTIVIRAL agents - Abstract
Abstract: A novel pathway of CD8+ T-cell activation by a previously established human Langerhans cell (LC)-like cell line, ELD-1 [Dendritic Cells 9 (1999) 41] is reported. ELD-1 cells possess LC-specific and dendritic cell (DC) lineage-specific markers including Birbeck granules. Intriguingly, ELD-1 cells stimulated interferon (IFN)-γ production by purified allogeneic CD8+ T cells in an IL-2- but not IL-12-dependent manner, but failed to stimulate CD4+ T cells due to their lack of HLA-DR, CD40, CD80, and CD86 expression. Comparing active and inactive subclones of ELD-1 cells revealed that CD70 was a key molecule determining stimulatory ability. This was confirmed by the ability of transfected CD70-encoding cDNA to confer stimulatory capacity on inactive subclones of ELD-1. Therefore, it is concluded that CD70 expressed on ELD-1 cells has a crucial role in stimulating IFN-γ production by CD8+ T cells through an alternative pathway which does not require CD4+ T-cell help or CD28–B7 interactions. [Copyright &y& Elsevier]
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- 2004
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44. Congenital self-healing reticulohistiocytosis: report of a case and review of the literature.
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Ricart, J., Jimenez, A., Marquina, A., and Villanueva, A.
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- *
HISTIOCYTOSIS , *LETTERER-Siwe disease , *HEALING , *SYMPTOMS , *LANGERHANS cells , *NEWBORN infants , *LANGERHANS-cell histiocytosis , *NON-langerhans-cell histiocytosis - Abstract
Unlabelled: Congenital self-healing reticulohistiocytosis (CSHRH), a rare disorder initially seen at birth or in the newborn period, is characterized by spontaneously involuting skin lesions. We present the case of a 12-d-old female who was born with many red-brownish nodules scattered over her entire body, and which regressed spontaneously.Conclusion: Although there are no clues to differentiate CSHRH from Letterer-Siwe disease, some signs and symptoms might enable us to reach an accurate diagnosis. A long-term follow-up to detect evidence of relapse or progression of the disease would be well advised. [ABSTRACT FROM AUTHOR]- Published
- 2004
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45. New mechanism for amino acid influx into human epidermal Langerhans cells: L-dopa/proton counter-transport system.
- Author
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Falck, Bengt, Bendsoe, Niels, and Ronquist, Gunnar
- Subjects
- *
DOPA , *LANGERHANS cells , *AMINO acids - Abstract
Abstract: We have characterized a stereospecific transport mechanism for L-dopa into human epidermal Langerhans cells (LCs). It is different from any other amino acid transport system. It is highly concentrative, largely pH-independent, and independent of exogenous Na[sup +] , glucose and oxygen, and fuelled by a renewable intracellular energy source inhibited by iodoacetate but not by arsenate. We propose that the mechanism is a unidirectional L-dopa/proton counter-transport system. We have recently demonstrated anaerobic glycolysis in human epidermis, which substantiates the need of proton pumps for resident LCs. The findings prompt a re-evaluation of the profound changes LCs undergo when exposed to oxygen in aerobic culture. L-dopa is not metabolized by LCs but can rapidly be dislocated to the intercellular space by certain extracellular amino acids, i.e. LCs can profit by L-dopa in a dualistic way, altogether a remarkable biological phenomenon. [ABSTRACT FROM AUTHOR]
- Published
- 2003
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46. Repeated irradiation with suberythemal ultraviolet B reduces the number of epidermal Langerhans cells.
- Author
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Ishitsuka, Yukiko, Masunaga, Takuji, Koide, Chiharu, and Arakane, Kumi
- Subjects
- *
ULTRAVIOLET radiation , *LANGERHANS cells , *DENDRITIC cells , *SKIN diseases , *DERMATOLOGY - Abstract
The influence of repeated low-dose ultraviolet B (UVB) radiation, to which we are exposed in daily life, has not been fully clarified, although the damage caused by exposure to high-dose UVB radiation has been well-studied in recent years. To investigate skin damage caused by repeated low-dose exposure, we evaluated the extent of injury to the Langerhans cells which are known to be involved in the cutaneous immune system. The backs of hairless mice were exposed to the following doses of UVB radiation: 100 mJ/cm2 once, 50 mJ/cm2 twice, 25 mJ/cm2 four times or 10 mJ/cm2 ten times. Skin specimens were taken for histochemical and electron microscopic examination 24 h after the final irradiation. Epidermis exposed to UVB radiation demonstrated a decrease in the number of Langerhans cells which showed less dendricity. The population of these cells in specimens exposed to repeated suberythemal doses was reduced to 40%, whereas exposure to a single high dose of UVB with the same energy resulted in a reduction of only 33%. These results indicate that repeated suberythemal doses injure Langerhans cells more than a single high-dose exposure. Furthermore, Birbeck granules in Langerhans cells of UVB-irradiated epidermis were reduced and tended to show shortening of their rod portion. The present study suggested that repeated challenge with suberythemal UVB radiation, to which we are all exposed in daily life, can cause substantial damage to Langerhans cells. [ABSTRACT FROM AUTHOR]
- Published
- 2003
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47. ГИСТИОЦИТОЗ ИЗ КЛЕТОК ЛАНГЕРГАНСА. КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ
- Subjects
молочная железа ,антигенпрезентирующие дендритные клетки ,гранулы Бирбека ,Langerhans cell histiocytosis ,Клетки Лангерганса ,antigen presenting dendritic cells ,Birbeck granules ,breast - Abstract
В статье описан случай гистиоцитоза из клеток Лангерганса, критерии диагностики, роль цитологического метода в установлении диагноза, Case report of Langerhans cell histiocytosis, diagnostic criteria, cytology value in final diagnosis.
- Published
- 2019
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48. A Case of Extensive Mass on the Scalp with Alopecia.
- Author
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Pomsoong C and Suchonwanit P
- Abstract
Competing Interests: The authors have no conflicts of interest to declare.
- Published
- 2022
- Full Text
- View/download PDF
49. Birbeck granules, worm-like bodies and 'lupus erythematosus virus'
- Author
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Hiroshi Fujiwara, Keiko Fujiwara, and Ken Hashimoto
- Subjects
Adult ,Inclusion Bodies ,Male ,Pathology ,medicine.medical_specialty ,Skin Neoplasms ,Lupus erythematosus ,integumentary system ,Birbeck granules ,fungi ,Histiocytes ,Dermatology ,Biology ,Cytoplasmic Granules ,Endoplasmic Reticulum ,medicine.disease ,Virus ,Microscopy, Electron ,parasitic diseases ,Immunology ,medicine ,Humans ,Facial Neoplasms - Abstract
The origin of worm-like bodies and their relationship with Birbeck granules is poorly understood. To clarify the origin of worm-like bodies and their relationship with Birbeck granules. Over 800 electron micrographs of histiocytic disorders and several appendage tumours were reviewed in order to check for worm-like bodies and Birbeck granules. Worm-like bodies were most often encountered in mildly- to moderately-proliferative histiocytic tumours. Birbeck granules were observed in more malignant conditions. Narrow endoplasmic reticulum (Nrer), which resembles worm-like bodies, was abundant in worm-like body-rich cells and coexisted with Birbeck rods. Nrer is thought to be one of the candidates that gives rise to worm-like bodies. An ultrastructural similarity exists between worm-like bodies (and octopus body formation) and the so-called "lupus erythematosus virus". The presence of Nrer is often concomitant with other organelle markers, and could be a candidate for the origin of worm-like bodies.
- Published
- 2016
50. Kinetic and Structural Studies of Interactions between Glycosaminoglycans and Langerin
- Author
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Jing Zhao, Robert J. Linhardt, Xinyue Liu, Chelsea Kao, Fuming Zhang, Quanhong Li, and Emily Zhang
- Subjects
Models, Molecular ,0301 basic medicine ,Glycan ,Langerin ,Protein Conformation ,Birbeck granules ,media_common.quotation_subject ,Plasma protein binding ,Biochemistry ,Dermatan sulfate ,03 medical and health sciences ,chemistry.chemical_compound ,Protein structure ,Sulfation ,Antigens, CD ,Humans ,Lectins, C-Type ,Internalization ,Glycosaminoglycans ,media_common ,integumentary system ,biology ,Heparin ,Kinetics ,Mannose-Binding Lectins ,030104 developmental biology ,chemistry ,biology.protein ,Protein Binding - Abstract
Langerin, a C-type lectin, is expressed in Langerhans cells. It was reported that langerin binds sulfated glycans, which is an important initial step for its role in blocking human immunodeficiency virus (HIV) transmission by capturing HIV pathogens and mediating their internalization into Birbeck granules for their elimination. It is fundamentally important to understand these interactions at the molecular level for the design of new highly specific therapeutic agents for HIV. Surface plasmon resonance (SPR), which allows for the real-time, direct, quantitative analysis of the label-free molecular interactions, has been used successfully for biophysical characterization of glycosaminoglycan (GAG)-protein interactions. In this study, we report kinetics, structural analysis, and the effects of physiological conditions (e.g., pH, salt concentration, and Ca(2+) and Zn(2+)concentrations) on the interactions between GAGs and langerin using SPR. SPR results revealed that langerin binds to heparin with high affinity (KD ∼ 2.4 nM) and the oligosaccharide length required for the interactions is larger than a tetrasaccharide. This heparin/heparan sulfate-binding protein also interacts with other GAGs, including dermatan sulfate, chondroitin sulfates C-E and KS. In addition, liquid chromatography-mass spectrometry analysis was used to characterize the structure of sulfated glycans that bound to langerin.
- Published
- 2016
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