Search

Langerhans’ cell histiocytosis is a collective term used to describe a group of enigmatic, proliferative disorders. The natural history of the disease varies from a slow, benign, localized, symptomatic bony or soft-tissue lesion, to a rapidly, progressive, widespread, multiple-organ disorder which is often fatal. Eosinophilic granuloma (EG) accounts for 60–70% of all cases of Langerhans’ cell histiocytosis and can present as solitary (50–75%) or multifocal defects in bone. It occasionally presents as a localized soft-tissue lesion. Multiple treatment options are available but the response is unpredictable. However, no studies show complete resolution following a conservative modality of treatment in the management of EG of condyle and ramus. We describe a case, treated with intralesional injections of triamcinolone acetonide (Tricort 40 mg/ml, 6 injections at weekly intervals over 6 weeks), leading to complete resolution of the lesion, along with a brief mention of the review of literature.

Background: Letterer–Siwe Disease (LSD) is one of the variants of Langerhans cell histiocytosis (LCH), which is considered as a rare disease that affects many systems in the body; it is characterized by monoclonal migration and proliferation of specific dendritic cells. The disease affects the bones and skin primarily, but can involve other organs as well, or appear as a multi-system disease leading to different clinical manifestations and eventually death. Summary: The authors present a case report of LSD in a two-year-old child from western Sudan, Messeria tribe, who is presented with one and a half-month history of fever, cutaneous ulcers, purprae, scaly crusted scalp, and pallor. His full blood count showed very low Hb with marked reduction of platelets. TWBC was normal. US showed hepatosplenomegaly with lymphadenopathy. A suspicion of sickle cell anemia and leukemia was suggested. He received treatment in his area in the form of antibiotics, skin care, blood transfusion and platelets aggregate without improvement. Patient was referred to Khartoum for further investigations and management. On presentation, a diagnosis of histiocytosis x was suggested depending on the clinical presentation of a general ill health in a child with purpurae, skin ulcers, and a scaly crusted scalp. A skin biopsy, bone marrow aspirate, and a skull x-ray were requested. Bone marrow aspiration showed hyper cellular BM with marked hemophagocytosis. Patient was admitted in a pediatric ward for further general investigations and blood transfusion, but he passed few days later before starting chemotherapy. Usually this is the prognosis of this rare and fatal aggressive form of histiocytosis x. Conclusion: A sick child with fever, anemia, hepatosplenomegaly, scaly scalp, and skin lesions should be investigated for LSD. Keywords: Langerhans cell histoctosis (LCH), Letterer–Siwe Disease (LSD), seborrheic dermatitis

Object The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease. Methods Forty-four patients with LCH who presented to a single pediatric neurosurgical department between 1976 and 2006 were retrospectively reviewed. Results This series included 29 boys and 15 girls, ranging in age from 2 months to 13 years, with a mean follow-up duration of 4.5 years. Twenty-seven patients (61%) had unifocal bone lesions, 12 (27%) had multifocal bone disease, 2 (5%) had solitary hypothalamic–pituitary axis lesions, and 3 (7%) had multiple organ involvement at presentation. Five (19%) of the 27 patients with unifocal bone disease and 4 (33%) of the 12 patients with multifocal bone disease had delayed development of new bone lesions during the follow-up period. The time to development of new bone lesions ranged from 1 month to 1 year. Two of the 3 patients with multiple-organ LCH died. Patient age ≤ 2 years at the time of initial presentation was a risk factor for both initial multifocality and eventual dissemination. In all patients with initial multifocal bone involvement or later dissemination of unifocal bone disease, LCH was controlled by chemotherapy, except for 2 who were treated by surgery alone. Three patients had histological evidence of spontaneous resolution of their lesions. Conclusions Patients with unifocal LCH can be effectively treated with surgery alone. Very young patients are more likely to have multifocal disease and disseminations, and will usually require chemotherapy to control their disease. Spontaneously regressing lesions need not be resected; however, a biopsy procedure can be performed for diagnostic purposes.

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.

Langerhans cell histiocytosis (LCH) is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. These syndromes are: eosinophilic granuloma, which is predominantly osseous or pulmonary; Hand-Schûller-Christian's disease, which involves multiple organ systems and, most typically, the skull base; and Letterer-Siwe's disease, the most severe disease manifestation, which typically involves the abdominal viscera. This article reviews our current understanding of Langerhans cell histiocytosis by discussing the history, histology, etiology, and treatment of the disease. It focuses on the radiographic findings and imaging modalities that are the most useful in disease diagnosis and management.

Langerhans cell histiocytosis (LCH) has diverse presentations which can bring it before all physicians regardless of specialty. A retrospective audit was undertaken at Westmead Hospital, Sydney, Australia, to ascertain the incidence, epidemiology and clinical features of patients with LCH over a 10-year period (1994-2004). A total of 12 patients was identified (six male, six female). Eleven patients had involvement of the skeletal system, three of the patients had pulmonary LCH and only one patient presented with soft tissue involvement (nose and antrum). Three patients had diabetes insipidus. Our results are consistent with that noted in the published literature and confirm the low incidence, diverse nature of presentation and the differing treatment strategies available for this rare and yet interesting condition.

Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, and Letterer-Siwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder which is usually sporadic. This report describes three cases in one family, who developed the disease a few years after their brother was found to be suffering from histiocytosis. All 3 patients had the same clinical manifestations: hyperthermia, eczematic rash, and swelling in skull, hand, and foot. X rays showed lytic areas in the skull and metacarp of fourth finger. Serology for EBV infection was negative. Infiltration of abnormal Langerhans cells histiocytes were demonstrated in bone biopsies. These patients were given chemotherapy. Case 1 (brother) died 1 year after chemotherapy, case 2 (girl) was given chemotherapy without success. She was given T-cell suppressor (cyclosporine), which induced remission, and case 3 was given chemotherapy, which was successful.

Introduction Atypical lymphoproliferative, histiocytic, and dendritic cell disorders often mimic their more common benign or malignant counterparts, and thus can easily be misdiagnosed. Although it may be challenging to identify these unusual disease entities precisely, doing so may significantly alter further evaluation, prognosis, and management of the patient. Multidisciplinary collaboration between clinicians and pathologists is often required to arrive at a definitive diagnosis. Critical to this process is obtaining adequate tissue, ideally through excisional lymph node biopsy. Although core-needle biopsy may sometimes yield a diagnosis, for many of these entities it will be inadequate. Fine needle aspiration has been demonstrated to be of little value in the diagnosis of lymphoproliferative disorders, as it does not provide an adequate assessment of tissue architecture. In cases where a biopsy is equivocal, additional biopsies may be necessary to establish a firm diagnosis. In this chapter, we will summarize the pathophysiology, presentation, diagnosis, prognosis, and treatment of atypical lymphoproliferative, histiocytic, and dendritic cell disorders. Three main categories of these disorders have been defined (Table 17.1). Benign conditions, such as Kikuchi's disease, often behave indolently and can usually be managed with observation. Histiocytic disorders exhibit a wide range of behavior, and may require more aggressive therapies. Atypical lymphoproliferative disorders can be polyclonal, such as Castleman'sdisease, or malignant, such as lymphomatoid granulomatosis, and are treated in a variety of different ways.

Among the several forms of Langerhans cell histiocytosis (LCH), Letterer-Siwe disease is the most acute disseminated multisystemic variant. The course of Letterer-Siwe disease is usually rapid and fatal. We report here a cases of Letterer-Siwe disease which showed a good response to chemotherapy. We also describe the unusual ultrastructural findings in the histiocyte-like cells in this case.

Langerhans cell histiocytosis is known to mimic many other conditions. We present two patients where anti-tubercular therapy was instituted when clinical and radiological features suggested tuberculosis. The correct diagnosis of histiocytosis was reached only on further work-up including immunohistochemistry following un-responsiveness to treatment. In retrospect it was felt that an inordinately high index of suspicion for tuberculosis, clinical and radiological overlap between the two entities, an urgency to start empirical therapy in a rapidly deteriorating patient and pathological similarities between epithelioid histiocytes and pathologic Langerhans cells led to the diagnostic and therapeutic errors. Pediatr Blood Cancer 2009;53:111–113. © 2009 Wiley-Liss, Inc.

Despite extensive literature on Langerhans’ cells histiocytosis, it is a poorly understood disease. Controversies exist with respect to its terminologies, origin, classification staging, and management. Recognizing their clinical relationship and pathological similarities, Lichtenstein, in 1953, grouped three conditions (namely, eosinophilic granuloma, Letterer-Siwe disease, and Hand-SchiillerChristian disease) under a general termhistiocytosis X.’ Because the lesions in each disease contain a common histological appearance, that is, the presence of Langerhans’ cells histiocytes and “small round cells” with a varying number of eosinophils, Lichtenstein proposed that these diseases were simply different expressions of the same basic disorder.2 The chronic form, which localized to bone as solitary or multiple lesions, was called eosinophilic granuloma. The disseminated chronic form, involving both skeletal and extraskeletal tissues, was termed Hand-SchiillerChristian disease. Letterer-Siwe disease was defined as the acute or subacute disseminated form of histiocytosis X.3 Letterer-Siwe disease is the most malignant of the three forms and is almost exclusively seen in infants. Libermann et al, in an extensive review, have suggested that both eosinophilic granuloma and Hand-Schiiller-Christian disease represent different expressions of

Various treatment modalities exist for Langerhans cell histiocytosis(LCH) due to its uncertain aetiology.. LCH patients are clinically divided into a single and multisystem group and each group warrants a different approach to treatment. However, histologically, CD Ia antigen and Birbeck's granules have been implicated in both types of the disease. This article therefore describes clinical presentations of different forms of the disease and the treatments contrived for them. The single system group is treated conservatively with either curettage of the localised lesion, intralesional steroid injection or in some cases radiotherapy. For the multisystem group, treatment consists of chemotherapeutic agents as either a single or combination therapy.

Background Langerhans cell histiocytosis (LCH) in adults first presenting in the skin is rare. Guidelines for staging, treatment and follow-up are lacking.Objectives To better define staging procedures, treatment results and clinical course in adult patients with LCH first presenting in the skin.Methods Eighteen adult patients with LCH first presenting in the skin were collected from five centres collaborating in the Dutch Cutaneous Lymphoma Group. Clinical records and (skin) biopsy specimens were reviewed and follow-up data were obtained. A literature search on adult patients with LCH presenting in the skin was performed.Results Staging procedures showed extracutaneous disease in three of 16 patients who were adequately staged. One patient had a histologically confirmed lytic LCH bone lesion, while two patients had a myelodysplastic syndrome. During follow-up two of 18 patients developed extracutaneous localizations of LCH. Five patients developed a second haematological malignancy, including (myelo)monocytic leukaemia (two cases), histiocytic sarcoma (one case), diffuse large B-cell lymphoma (one case) and peripheral T-cell lymphoma (one case). Review of the literature revealed six other adult patients with a second haematological malignancy preceding or following a diagnosis of LCH.Conclusions The results of the present study suggest an increased risk of a second haematological malignancy in adult patients with LCH presenting in the skin. Extensive staging at presentation and long-term follow-up are therefore warranted in such patients.

Langerhans cell histiocytosis is a rare disorder that occurs when there are too many of type of white blood cell called a Langerhans cell. We describe a 3-months infant girl who developed wide spread skin lesions since she was 4 days of age. . The diagnosis of Langerhans cell histiocytosis was confirmed by histopathology. We report this case as a first Langerhans cell histocytosis to be reported in an infant.

From the Department of Radiology, Division of Neuroradiology,a and the Department of Dermatology,b Indiana University Medical Center; and the Department of Oral Surgery Medicine and Pathology, Indiana University School of Dentistry.c Reprint requests: Ginat W. Mirowski, DMD, MD, Department of Dermatology, 550 N University Blvd, Suite 3240, Indianapolis, IN 46202-6267. E-mail: gmirowsk@iupui.edu. J Am Acad Dermatol 2001;44:509-11. Copyright © 2001 by the American Academy of Dermatology, Inc. 0190-9622/2001/$35.00 + 0 16/1/109304 doi:10.1067/mjd.2001.109304 LANGERHANS CELL HISTIOCYTOSIS Langerhans cell histiocytosis has replaced the term histiocytosis X, originally coined by Lichtenstein in 1953 to link 3 related clinical entities of unknown origin, characterized by Langerhans cell proliferation (Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma).

Background and importance Langerhans cell histiocytosis (LCH) is an uncommon disease, usually affecting the cranium and peripheral bones. We present a rare case of isolated optic chiasm involvement by LCH to highlight the importance of considering LCH in the differential diagnosis of optic chiasm lesions. Clinical presentation A 71-year-old woman presented with a 6-week history of worsening peripheral vision, headaches, weakness, cold sensitivity, and fatigue. She was found to have dense bitemporal hemianopsia. Magnetic resonance imaging revealed a 2-cm lesion, contrast enhancing on T1 and bright on T2 signal, involving the optic chiasm but not the pituitary gland. Preoperative considerations included optic nerve glioma, choristoma of the stalk, sarcoid, hypothalamic glioma, and Langerhans cell histiocytosis. The patient underwent a right subfrontal craniotomy for biopsy of the lesion. The optic chiasm was grossly enlarged with no tissue external to the chiasm. A midline incision was made in the lamina terminalis, and multiple biopsies were taken of firm fibrous material. Histologically, the tumor was characteristic for LCH and included a mixture of histiocytes with features of Langerhans cells, eosinophils, small lymphocytes, macrophages, neutrophils, and plasma cells. Conclusion LCH is a rare disease, generally affecting bone, skin, lymph nodes, and in more severe cases, visceral organs. LCH involving the optic pathways is a rare condition that should be included in the differential for adults with mass lesions involving the orbit, eye, optic nerve, or chiasm. Future clinical and basic science research is needed to better understand LCH, its molecular origin, and its growth pattern.

Langerhans cell histiocytosis (LCH) is a rare clonal disorder of unknown etiology and characterized by the proliferation of dendritic cells. LCH most commonly involves the bone followed by the skin and the lymph nodes. Recently, only a few cases of LCH with predominant lymph node involvement have been diagnosed by fine-needle aspiration cytology (FNAC). A 2-year-old boy presented with generalized lymphadenopathy, fever, and cough. The patient had hepatosplenomegaly, anemia, and lytic lesions in the skull. FNAC from the largest submandibular lymph node showed features of LCH. The large cells of LCH showed positive immunostaining for S-100 protein on FNAC smears. Later, lymph node biopsy and immunohistochemistry against S-100 protein and CD1a confirmed the diagnosis of LCH. The patient was treated with chemotherapy and he is under regular follow-up. This case report highlights the importance of FNAC as a rapid and accurate investigation in the diagnosis of lymph node predominant LCH.

Langerhans cell histiocytosis (LCH) is a very rare disorder, and usually considered a disease of childhood. The adult form of LCH is even rare, and in some case may be considered to represent delayed presentation of a pathologic process beginning in childhood. This case report highlights the range of presentations of LCH possible even in a single patient, and supports the contention that some adult LCH cases may be due to delayed presentation of childhood LCH.

The histiocytic disorders are defined as disorders due to an abnormal accumulation of cells of the mononuclear phagocytic system (MPS), consisting of dendritic cells (DCs) and macrophages. They comprise a wide variety of diverse conditions that affect both children and adults, and have been difficult to classify and to treat. Figure 1.1 is a simplified schematic representation of the development of various components of the MPS from the primitive CD34 hematopoietic stem cell. 1

Eosinophilic granuloma is a localised painful lesion of either of skull bone, ribs or pelvis with a good prognosis. Calvarial swellings have an affinity to temporal bone. Recently eosinophilic granuloma has been grouped along wiht Letterer Siwe disease and Hand Schuller Christian disease under a common heading called Histiocytosis-X.

Objectivo: Descrever os aspectos clínico-radiológicos da Histiocitose de Células de Langerhans (HCL) numa série de doentes. Material e Métodos: Reviram-se 20 casos de HCL, nove com diagnóstico segundo os actuais critérios anátomo-patológicos (positividade para CD1a e/ou presença de grânulos de Birbeck em microscopia electrónica), outros nove apenas com critérios morfológicos, e dois casos com diagnóstico patológico estabelecido fora da instituição, não susceptível de aferição anátomo- patológica. Resultados: A razão sexo masculino:feminino foi 4:1 e a média de idades de apresentação da doença 8 anos (variação: 2 meses - 44 anos). Os quatro órgãos mais frequentemente envolvidos foram, por ordem decrescente: osso, gânglios linfáticos, pele e hipófise. Dois doentes tinham doença unifocal óssea, oito apresentavam doença multifocal-unissistémica, em sede óssea ou ganglionar, e dez doença multissistémica. As lesões líticas foram o achado radiológico mais frequente, distribuindo-se predominantemente na calote craniana, ossos ilíacos e fémures. As alterações pulmonares constituiram achado minoritário. Conclusão: A série em estudo incluiu uma proporção grande de casos com gravidade clínica (10 doentes com HCL multissistémica), tendo-se observado em número relativamente restrito a forma menos grave da doença, correspondente ao clássico granuloma eosinófilo. A aparência das alterações radiológicas foi contudo similar à descrita na literatura. Objective: To describe the clinical and radiological features of Langerhans Cell Histiocytosis (LCH) in a series of patients. Material And Methods: We reviewed 20 cases of LCH, nine with diagnosis by the current pathological criteria (positivity for CD1a and/or presence of Birbeck granules identified at ultrastructural examination), and nine only with morphological criteria. In the other two cases, the pathological diagnosis was made outside our institution and could not be reviewed. Results: Male:female ratio was 4:1 and the average age of presentation of the disease was 8 years (range: 2 months - 44 years). The four most affected organs were, in decreasing order: bone, lymph nodes, skin and hypophysis. Two patients had unifocal bone disease, eight multifocal unisystem involvement in the bone or lymph nodes, and ten presented multisystem LCH. Lytic lesions were the most common radiological finding, affecting mainly the skull, iliac bones and femurs. Pulmonary lesions were rather infrequent. Conclusion: This series included a large proportion of severe cases (10 patients with multisystem LCH), while the mildest form of the disease, classically designated as eosinophilic granuloma, was observed only in a few patients. The appearance of the radiological findings was however similar to the described in the literature.

Submitted by Teresa Margarida Cunha (tmargarida@gmail.com) on 2016-07-24T07:39:01Z No. of bitstreams: 1 2004-Histiocitose de células de Langerhans- Um estudo de 20 casos.pdf: 984012 bytes, checksum: 056bab175f3f3dccbb90555e0a56b516 (MD5) Approved for entry into archive by Raquel Truta (comum@rcaap.pt) on 2016-11-25T14:15:41Z (GMT) No. of bitstreams: 2 1008624248408986.zip: 982283 bytes, checksum: 06a427dae772fa7bc76f62fe1d21e6d3 (MD5) 2004-Histiocitose de células de Langerhans- Um estudo de 20 casos.pdf: 984012 bytes, checksum: 056bab175f3f3dccbb90555e0a56b516 (MD5) Made available in DSpace on 2016-11-25T14:15:41Z (GMT). No. of bitstreams: 2 1008624248408986.zip: 982283 bytes, checksum: 06a427dae772fa7bc76f62fe1d21e6d3 (MD5) 2004-Histiocitose de células de Langerhans- Um estudo de 20 casos.pdf: 984012 bytes, checksum: 056bab175f3f3dccbb90555e0a56b516 (MD5) Previous issue date: 2004

A 15-month-old girl with Langerhans cell histiocytosis (LCH), Letterer-Siwe disease, was referred to our hospital in 1984. Whilst on treatment with cytotoxic drugs, a perirenal mass was detected and hydronephrosis became evident when she was 29 months old. Percutaneous nephrostomy tubes were placed in the pelvis, bilaterally and replaced every 6 months. The mass was not completely controlled and chronic pyelonephritis continued. Biopsy of the mass convoluted kidney hilus revealed histiocytic invasion. Although multiple organ systems are involved in LCH and abdominal malignant tumours may be accompanied by hydronephrosis, to our knowledge, this is the first case report of abdominal LCH and the ensuing hydronephrosis. Percutaneous nephrostomy tubes proved useful, but more convenient, less painful and infection-limited approaches need to be designed.

A case of Langerhans Cell Histiocytosis (LCH) is reported in 1½ years old boy. He had seborrhoeic dermatitis like condition of scalp, papular lesions with purpura typical of Letterer-Siwe disease associated with constitutional symptoms, hepatosplenomegaly, jaundice, anaemia and thrombocytopenia. Peripheral blood film and bone marrow examination showed presence of LCH cells.

Histiocytosis is a rare disease of unknown aetiology which commonly affects the head and neck region. In the ear it can closely mimic acute and chronic ear disease leading to a confusion in diagnosis. It can be easily misdiagnosed unless a high index of clinical suspicion is maintained. We present a case of Letterer Siwe disease of the temporal bone which presented with bilateral mastoid abscesses.

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which Langerhans's cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry findings suggested diagnosis of LCH.

Purpose: We report the unrecognized association of Langerhans cell histiocytosis (LCH) with partial DiGeorge syndrome. Patient and Methods: A 7-week-old infant with endocrine and immunologic characteristics of DiGeorge syndrome displayed multisystem involvement of Letterer-Siwe disease at birth. Results: Despite vigorous medical support and chemotherapy, she died at 9 months of age with multisystem failure. Conclusion: This case supports the role of the thymus in the pathogenesis of LCH.