Search

The current report examines the evolution of the concepts of immunocytoma and pseudolymphoma in a historical perspective, paying special attention to their evolvement into the groups of marginal-zone lymphoma and cutaneous MALT-lymphoma. It also examines the current conception of the existence of at least two types of cutaneous MALT-lymphomas and their relation to the duality immunocytoma/pseudolymphoma from the old literature.

A 39-year-old man presented with an annular clearly palpable erythema on the décolleté. Based on clinical and histopathological findings, palpable migratory arciform erythema was diagnosed. This skin condition is classified as a rare type of T-cell pseudolymphoma. It is still a matter of debate whether palpable migratory arciform erythema is a specific entity or a clinical variant of lymphocytic infiltration of the skin (Jessner-Kanof). Topical corticosteroids or oral antibiotics are generally used. In our patient, UV-A1 therapy led to a complete regression of the lesions.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is characterized by multiple angiomatous papules on the hands and feet in children. Here, we report a case of APACHE in a female patient followed up from 13 to 19 years of age with a dark red lesion on the center of the dorsum of the right thigh. Histologically, vacuolar alteration and exocytosis of lymphocytes, and specific dense cellular infiltration beneath the epidermis to the reticular dermis were found. On immunolabeling study, the lesion vessels were found to be positive for both the lymphatic endothelium-specific marker podoplanin and blood vessel-specific marker CD34. These findings suggested that APACHE is a type of vascular malformation.

Tattoo-induced pseudolymphoma is a cutaneous inflammatory response, the pathogenesis of which is still unknown. The objective of the present work was to find a possible causal relationship between pseudolymphomatous reactions on the red areas of tattoos and the metals contained in tattoo pigments and skin biopsies. Three individuals with cutaneous lesions on the red areas of tattoos were observed. Clinical and immunohistochemical examinations of the lesions were performed, and the concentrations of Cd, Co, Cr, Hg, Ni and Pb were measured in pigments and biopsy samples. Pseudolymphomas in the red areas were diagnosed in all three cases; one showed the prevalence of B-lymphocytes, whereas the other two showed a prevalence of T cells with a lichenoid pattern. Patch tests were negative. Corticosteroid therapy was ineffective. Cadmium, Co, Hg and Pb in the pigments were probably present as impurities, whereas Cr and Ni were the main components. Chromium and Ni had the highest concentrations, also in the biopsy samples. Permanent tattoos appear to be unsafe, considering the increasing number of diagnosed pseudolymphomas. It can be excluded that Hg was responsible for the reactions, given that the concentration in the red dyes and biopsies was very low. Significant levels of Cr and Ni should be considered as the causes of possible dermal reactions.

Complications of tumorigenesis by a lacrimal silicone plug are rarely reported and most of them were cases of granuloma due to chronic inflammation. The authors present a case of a 57-year-old healthy woman complaining of lower eyelid swelling and protruding mass from lower punctum who had undergone insertion of a lacrimal silicone plug. Surgical mass resection was performed and initial histopathology revealed lymphoid hyperplasia. Additional immunophenotypic markers CD3, CD20, and BCL2 showed reactive lymphoid hyperplasia. It has a histopathologic significance in that it can mimic lymphoma and should be distinguished from malignancy.

Disseminated miliarial-type lymphocytoma cutis is a rare clinicopathologic subtype of lymphocytoma, characterized by numerous translucent micropapules and located on the head and neck. We describe here the clinical, histological and immunological features of miliarial-type perifollicular B-cell pseudolymphoma in two women aged 32 and 49 years presenting with numerous 1-2 mm translucent papules on the head and neck. Microscopic examination revealed features typical of a miniature lymphocytoma cutis with a superficial nodular infiltrate housing small well-circumscribed germinal centers containing tingible body macrophages and surrounded by a distinct mantle zone. The immunohistological profile was also typical of lymphocytoma cutis, and polymerase chain reaction analysis of the IgH gene rearrangement did not show any clonal B-cell population. The lesions resolved spontaneously in one case and improved after treatment with hydroxychloroquine in the second patient. The presentation of this subtype of lymphocytoma is a formidable clinical diagnostic challenge. Rosacea, sarcoidosis, democidiosis, lupus miliaris disseminatus faciei and steroid acne are easy to exclude on the basis of histological profile. However, the miliarial form of primary cutaneous center lymphoma is difficult to rule out. Dermatologists and dermatopathologists should be aware of this unusual form of lymphocytoma cutis, which can be misleading during clinical diagnosis., (Copyright © 2012 John Wiley & Sons A/S.)

The term pseudolymphoma refers to a heterogeneous group of benign reactive T-cell or B-cell lymphoproliferative processes of diverse causes that simulate lymphoma clinically and histologically but usually undergo spontaneous remission. Its pathogenesis is still unclear. The prognosis is good although some evidence suggests that pseudolymphoma may progress to lymphoma. Pseudolymphoma of the urinary tract is extremely rare. We herein report a case of pseudolymphoma of the renal sinus in a 70-year-old man, associated with a high grade urothelial carcinoma of the bladder and to a prostatic adenocarcinoma (Gleason score 6). A brief review of the literature is included. The kidney showed a well-defined, whitish soft mass which involved the renal sinus. Microscopically, the lesion of the renal sinus consisted of a proliferation of small to medium size lymphocytes (CD20 positive and Bcl-2 negative) sometimes arranged in hyperplastic follicular structures. The diagnosis was confirmed by molecular studies which showed an oligopolyclonal IgH rearrangement. To the best of our knowledge, this is the second case of pseudolymphoma with a complete molecular characterization ever described in the renal sinus and the first one associated with multiple urogenital carcinomas.

Aim: To describe the clinical and histopathological features of ocular reactive lymphoid hyperplasia in children, and review the literature regarding this entity., Materials and Methods: In this retrospective, interventional case series, a chart review was performed of three patients diagnosed with reactive lymphoid hyperplasia. Details of clinical presentation, ocular and systemic examination findings, management and subsequent course were noted., Results: Three children, aged 9-14 years presented with ocular adnexal masses (two unilateral and one bilateral) with 7-12 months duration. Ocular examination revealed discrete nasal conjunctival masses in two patients, and bilateral eyelid fullness and conjunctival chemosis in the third patient. Systemic evaluation and laboratory tests were normal in all patients. Orbital imaging showed lacrimal gland enlargement in one patient. Histopathological evaluation with immunohistochemical markers established the diagnosis of reactive lymphoid hyperplasia. Two patients underwent surgical excision with complete resolution. All patients have remained stable and at their last follow-up have showed no evidence of recurrence, transformation, or systemic involvement., Conclusion: Reactive lymphoid hyperplasia, though uncommon in children, can have a favorable outcome with timely intervention.

Extra nodal marginal zone B-cell lymphoma (EMZL) of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. The pathogenesis of conjunctival EMZL remains unknown. We describe an unusual case of EMZL arising from reactive lymphoid hyperplasia (RLH) of the conjunctiva. A 35-year-old woman had fleshy salmon-pink conjunctival tumors in both eyes, oculus uterque (OU). Specimens from conjunctival tumors in the right eye, oculus dexter (OD), revealed a collection of small lymphoid cells in the stroma. Immunohistochemically, immunoglobulin (Ig) light chain restriction was not detected. In contrast, diffuse atypical lymphoid cell infiltration was noted in the left eye, oculus sinister (OS), and positive for CD20, a marker for B cells OS. The tumors were histologically diagnosed as RLH OD, and EMZL OS. PCR analysis detected IgH gene rearrangement in the joining region (JH) region OU. After 11 months, a re-biopsy specimen demonstrated EMZL based on compatible pathological and genetic findings OD, arising from RLH. This case suggests that even if the diagnosis of the conjunctival lymphoproliferative lesions is histologically benign, confirmation of the B-cell clonality by checking IgH gene rearrangement should be useful to predict the incidence of malignancy.

Borrelial lymphocytoma (BL) is a rare cutaneous manifestation of Lyme borreliosis. Epidemiological data show that BL is more common in children than in adults. It presents as a single bluish-red swelling located on the earlobe in children, near the nipple in adults. In our paper, we present a case of a pregnant woman with BL, which appeared after tick bite and disappeared completely after antibiotic therapy. The aim of the paper was to emphasize that in tick-borne disease endemic areas BL should be taken into consideration in cases of skin lesions., (Copyright © 2012 Elsevier GmbH. All rights reserved.)

The clarification of enlarged lymph nodes is a common issue in clinical routine. By now, open surgery with complete lymph node extirpation, followed by histopathology, is considered as standard. We investigated the value of fine needle aspiration (FNA) and core needle biopsy (CNB) when supporting the conventional morphology by immunotyping. In total, 101 lymph nodes (reactive, n = 19; lymphoma, n = 46; metastatic, n = 36) were examined. CNB specimens were sufficient for unequivocal diagnosis by histopathology in 95 %. The FNA cytology allowed a correct diagnosis in 49 %. When supported by immunocytology, the success rate improved to 72 %. By accepting "suspicious of" as correct diagnosis, the ratio increased to 91 %. Additional use of flow cytometry in 46 samples minimized the "suspicious of" diagnoses and increased the proportion of unequivocal diagnoses in FNA specimens to 87 %. Flow cytometry allowed a correct subtyping in 20 of 21 B cell lymphoma but recognised only one of five Hodgkin lymphoma. All eight reactive samples were correctly diagnosed by flow cytometry. In summary, CNB allows a reliable clarification of an unclear lymphadenopathy. FNA is a powerful first diagnostic approach, especially if cytology is supported by immunocytology. The most substantial contribution of flow cytometry in FNA is the discrimination between reactive lymphadenopathy and B cell lymphoma.

The gastrointestinal tract is the most common extranodal site of lymphoma involvement. Although B-cell lymphomas are by far the most frequent type found in this location, gastrointestinal lymphomas are a diverse group of neoplasms, many of which are characterized by distinctive clinicopathologic settings. Diffuse large B-cell lymphoma and marginal-zone lymphoma of mucosa-associated lymphoid tissue are commonly encountered, but other less-common entities can pose diagnostic challenges, mimicking both benign, reactive conditions and each other. We describe several different lymphoma subtypes, with a focus on frequently encountered challenges in differential diagnosis.

Pulmonary lymphoproliferative disorders (LPD) are characterised by abnormal proliferation of indigenous cell lines or infiltration of lung parenchyma by lymphoid cells. They encompass a wide spectrum of focal or diffuse abnormalities, which may be classified as reactive or neoplastic on the basis of cellular morphology and clonality. The spectrum of reactive disorders results primarily from antigenic stimulation of bronchial mucosa-associated lymphoid tissue (MALT) and comprises three main entities: follicular bronchiolitis, lymphoid interstitial pneumonia and (more rarely) nodular lymphoid hyperplasia. Primary parenchymal neoplasms are most commonly extranodal marginal zone lymphomas of MALT origin (MALT lymphomas), followed by diffuse large B-cell lymphomas (DLBCLs) and lymphomatoid granulomatosis (LYG). Secondary lymphomatous parenchymal neoplasms (both Hodgkin and non-Hodgkin lymphomas) are far more prevalent than primary neoplasms. Acquired immune deficiency syndrome (AIDS)-related lymphoma (ARL) and post-transplantation lymphoproliferative disorder (PTLD) may also primarily affect the lung parenchyma. Modern advances in treatments for AIDS and transplant medicine are associated with an increase in the incidence of LPD and have heightened the need to understand the range of imaging appearance of these diseases. The multidetector CT (MDCT) findings of LPD are heterogeneous, thereby reflecting the wide spectrum of clinical manifestations of these entities. Understanding the spectrum of LPD and the various imaging manifestations is crucial because the radiologist is often the first one to suggest the diagnosis and has a pivotal role in differentiating these diseases. The current concepts of LPD are discussed together with a demonstration of the breadth of MDCT patterns within this disease spectrum.

The authors report 2 cases of cutaneous pseudolymphoma that occurred in 2 young adult patients who referred for relentlessly growing nodules that appeared within 4 to 5 months after the application of a mercury-based tattoo. Systemic symptoms were not present and there was no evidence of lymph node enlargement. Clinically, both lesions were limited to the red, mercury-based areas of the tattoo. Microscopic examination featured a dense cellular infiltrate composed of polytypic T cells in the upper to mid-dermis, coupled with focal interface tissue reaction. Scattered macrophages contained finely granular particles in their cytoplasm. In addition, extracellular pigment particles were also recognized. Collections of epithelioid macrophages were present in both cases and were reminiscent of epithelioid granulomas. This study confirms evidence that, among skin diseases featuring a dense lymphoid infiltrate, cutaneous pseudolymphoma secondary to tattooing is a rare but not exceptional source of diagnostic challenges.

Objective: To study the clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT)., Methods: The clinical and pathologic findings were evaluated in 3 cases of biopsy confirmed thymic MALT lymphoma. The clincopathologic features, treatment and prognosis were discussed and literatures reviewed., Results: One male and two female patients presented with asymptomatic mediastinal masses with a history of Sjögren syndrome. They were aged 36, 35 and 41 years respectively, and only one patient had B symptoms. Grossly, all three tumors were encapsulated and had multiple variable-sized cysts on cut-surface. Histopathologically, the normal thymic lobular architecture was effaced by abnormal dense lymphoid infiltration. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding Hassall's corpuscles and epithelial cyst lining. All cases showed apparent plasmacytic differentiation. Immunohistochemically, the tumor cells were positive for CD20, CD79a, bcl-2 and negative for CD3, CD5, cyclin D1, CD43, CD10, bcl-6, and CD23. The plasma cells showed kappa light chain restriction. Immunoglobulin heavy chain rearrangement in three cases was confirmed by PCR. All patients were at early stage and received routine chemotherapy with or without radiotherapy after surgical removal. All patients achieved complete remission with 24, 18 and 3 months follow-up, respectively., Conclusions: Primary thymic MALT lymphoma may be a rare distinctive lymphoma. It can be diagnosed by HE and immunohistochemical study and should be differentiated from reactive lymphoid proliferation, other types of lymphoma and mediastinal thymoma.

This case report describes a 10-year-old horse that developed multiple dermal papules over the right masseter area following removal of a tick from the same site 3 months earlier. Histological examination of a biopsy from a papule was suggestive of either a T-cell-rich B-cell lymphoma or cutaneous lymphoid hyperplasia, a form of pseudolymphoma sometimes associated with a tick bite. Positive serological testing and PCR of the biopsy sample for Borrelia in conjunction with immunohistochemical testing of the skin biopsy, the clinical history and response to treatment with doxycycline strongly supported the diagnosis of Borrelia-associated cutaneous pseudolymphoma., (© 2011 The Authors. Veterinary Dermatology. © 2011 ESVD and ACVD.)

Objective: To evaluate the value of diffusion-weighted imaging (DWI) in the differentiation of axillary inflammatory hyperplastic and metastatic lymph nodes., Methods: Forty female New Zealand white rabbits were divided randomly into 2 groups (n = 20 each). And the animal models of axillary inflammatory hyperplastic and metastatic lymph nodes were established. All successfully implanted models received conventional magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI) examinations. The features of signal intensity and shapes of lymph nodes were observed in two groups. The sizes of lymph nodes were measured on the selected axial T(2)WI. The signal intensity and appearance diffusion coefficient (ADC) value of lymph nodes and dorsal muscle at the same slice were measured on the selected T(2)WI, DWI and ADC map respectively. The relative signal intensity (rSIT(2)WI, rSIDWI) and relative ADC (rADC) value of lymph nodes to dorsal muscle were calculated and compared. The diagnostic efficacy of differentiating benign and malignant lymph nodes was analyzed with rADC value through the receiver operating characteristic curve. The correlation between rADC value and cell density was assessed with pathological findings as reference standard., Results: The differences of size, rSIT(2)WI and rSIDWI were not statistically significant between two groups. The rADC value of inflammatory lymph nodes was greater than that of metastatic lymph nodes (0.91 ± 0.14 vs 0.64 ± 0.18). Significant difference existed (t = 3.879, P = 0.03). But there was a little overlap between two groups. With 0.78 as the diagnostic threshold of rADC value, the sensibility and specificity was 86.2% and 74.4% respectively. The correlation between rADC value and cell density of inflammatory hyperplastic and metastatic lymph nodes was significantly inverse (r = -0.53, P = 0.003)., Conclusions: As compared with the routine MRI sequence, rADC value has a higher diagnostic efficacy in the differentiation of benign and malignant lymph nodes. Cell density may be the most important influencing factor for the differences of rADC value between two groups of lymph nodes.

Objective: To study the dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) features of VX2 rabbits carcinoma metastatic and inflammatory lymph nodes., Methods: Twenty healthy adult New-Zealand white rabbits were divided into metastatic lymph node group and inflammatory lymph node group randomly. There were ten rabbits in each group. All 20 rabbits underwent DCE-MRI. Peak enhancement, time to peak, maximum slope and signal intensity versus time curves were generated from each node individually., Results: DCE-MRI were obtained for nine cases of metastatic lymph nodes and nine cases of inflammatory lymph nodes. The signal intensity versus time curves of metastatic lymph nodes were appeared as rapid ascending and plateauing, and peak enhancement, time to peak, maximum slope of metastatic lymph nodes group were 284% ± 125%, (118 ± 47) s and (6.5 ± 2.7)%/s, respectively. The signal intensity versus time curves of inflammatory lymph nodes were appeared as insisting ascending, and peak enhancement, time to peak, maximum slope of inflammatory lymph nodes group were 199% ± 109%, (143 ± 40) s and (3.6 ± 1.5)%/s, respectively. There was significantly higher peak enhancement, shorter time to peak and higher maximum slope in the metastatic lymph nodes group compared with the inflammatory lymph nodes group (P < 0.05)., Conclusions: DCE-MRI can accurately reflect the hemodynamic characteristics of VX2 rabbits neoplasm metastatic lymph nodes and inflammatory lymph nodes, and can differentiate VX2 rabbits neoplasm metastatic lymph nodes from inflammatory lymph nodes.

Objectives: To evaluate the clinical significance of HMGB1 expression in T-cell lymphoma., Methods: Immunohistochemical staining for HMGB1 and survivin was performed with specimens from 120 cases of T-cell lymphoma and 40 cases of reactive lymphoid hyperplasia with antibodies against human HMGB1 and survivin., Results: The expression of HMGB1 and survivin was significantly higher in tissues of T-cell lymphoma than in reactive lymphoid hyperplasia. Positive expression of HMGB1 and survivin was observed in 63.7% (65/102) and 61.8% (63/102) of T-cell lymphoma cases, respectively. While was associated with gender, age, and tumor location, significant correlations with malignancy and clinical stage were observed. Spearman rank correlation analysis revealed that the expression of HMGB1 and survivin was positively correlated in T-cell lymphomas (P<0.01)., Conclusions: Expression of HMGB1 and survivin in T-cell lymphomas is significantly associated with malignancy and clinical stage, but not with gender, age and tumor location. Elevated expression of HMGB1 may be an important biomarker for the development and progression of T-cell lymphoma.

Lymphoma-like lesion (LLL) of the female genital tract is an older term in the literature that describes a florid reactive lymphoid proliferation that can be misinterpreted as lymphoma. Multiple causes of LLL have been suggested but most cases remain unexplained. We describe the clinicopathologic features of 6 patients with LLL involving the uterine cervix. Five patients presented with abnormal Papanicolaou test (Pap smear), and 3 patients had a biopsy procedure performed prior to detection of LLL in a loop electrosurgical excision procedure (LEEP). In each specimen, surface epithelial erosion was associated with a superficial, polymorphous lymphoid infiltrate with numerous scattered large cells, without cellular necrosis or sclerosis. Squamous dysplasia was present in 4 patients. Immunohistochemical studies revealed a mixed population of B- and T-lymphoid cells. T-cells were more numerous but B-cells and formed aggregates or sheets in areas. The large cells were predominantly B-cells positive for CD20 and negative for CD3 in all cases. CD30 was positive 3 cases, and Epstein-Barr virus-encoded RNA was positive in 3 cases. Assessment for clonality in 1 patient using polymerase chain reaction (PCR) methods revealed monoclonal immunoglobulin heavy chain (IgH) gene rearrangements. At last clinical follow-up there was no evidence of progressive or systemic disease. We conclude that LLL of the cervix has a number of etiologies and that a prior surgical procedure, present in 3 patients in this study, is another possible etiology. As has been reported by others, monoclonal IgH gene rearrangements can be detected in this entity which has a benign clinical course., (Copyright © 2012 Elsevier Inc. All rights reserved.)

A 13-year-old boy presented with asymptomatic, bilateral, nasal bulbar conjunctival lesions. Excisional biopsy of the larger lesion showed a mature, mixed lymphocytic infiltrate with germinal centers, consistent with a diagnosis of benign reactive lymphoid hyperplasia, a rare condition in children., (Copyright 2011, SLACK Incorporated.)

Synucleins are small soluble proteins found in normal brain that facilitate rapid release of neurotransmitters. α-synuclein is a major component of the Lewy body of neurodegenerative diseases and γ-synuclein is a marker of aggressive carcinomas. As the role of γ-synuclein has not yet been investigated in the lymphoid system, we immunohistochemically stained normal lymphoid organs, lymph nodes with reactive lymphoid hyperplasia, and malignant lymphomas. The anti-γ-synuclein antibody strongly stained the follicular dendritic cell (FDC) meshworks and vascular and lymphatic endothelial cells in reactive lymphoid tissues, in B-cell lymphomas with a nodular pattern, and in angioimmunoblastic T-cell lymphomas. There were no γ-synuclein-positive FDC meshworks in B-cell or T-cell lymphomas with a diffuse pattern. This is in contrast to CD21, which only stained the arms of the FDCs; γ-synuclein highlighted both the long slender cellular processes and the cell body, thereby clearly demonstrating the number of individual FDCs. In addition, γ-synuclein was strongly expressed by the neoplastic counterpart of reactive FDCs (FDC sarcoma) and by the neoplastic counterparts of normal lymphatic and vascular endothelial cells (Kaposi sarcoma, hemangioma, and angiosarcoma). Only a few spindle cell neoplasms (SSNs) derived from smooth muscle, peripheral nerve, or gastrointestinal stroma expressed γ-synuclein; however, γ-synuclein was not expressed by 11 other types of SSNs tested. These results suggest that γ-synuclein is a promising new adjunct marker for identifying reactive FDCs and for diagnosing FDC sarcoma and benign and malignant vascular tumors.

Reactive lymphoid hyperplasia (RLH) is a rare, benign, lymphocytic tumour-like lesion reported in various organs. It has been previously identified in 18 cases in the English-language literature, but only 5 of them were related to oncological disease. No previous cases have been described of RLH occurring in ovarian cancer patients. We describe a case of hepatic RLH which developed in a patient treated for ovarian cancer 11 years previously. Radiological features on computed tomography (CT) scan and PET-CT (positron emission tomography-computed tomography) were strongly suggestive of oncological disease, in contrast to magnetic resonance imaging (MRI); the volume increment of the nodular lesion and the rise in carbohydrate antigen 125 corroborated the hypothesis of malignancy. The patient was subjected to resection of the 7th segment of the liver and the final histological report showed RLH. RLH should be considered in the presence of hepatic lesions in suspected ovarian cancer recurrence. Imaging techniques should be thoroughly investigated to exclude tumor recurrence promptly, in order to avoid unnecessary surgery.

Benign lymphoid hyperplasia (pseudolymphoma) has been reported in the skin, lungs, orbit, and gastrointestinal tract, but only rarely in soft tissues. These lesions mimic lymphoma both clinically and histologically. We describe a case of a pseudolymphoma of the deep soft tissues of the lower extremity. The lesion was composed of nonencapsulated lymphoid tissue with involvement of adjacent fat and connective tissues and multiple variably sized well-polarized germinal centers. Immunohistochemical staining, flow cytometry, chromogenic in situ hybridization for κ/λ light-chain restriction, and polymerase chain reaction for T- and B-cell gene rearrangements all revealed a polyclonal population of T and B cells, consistent with a benign reactive process. So far as we know, pseudolymphoma of the deep soft tissues has been described only once previously in the medical literature., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We report a rare case of duodenal pseudolymphoma without any symptoms. The lesion located in front of the head of the pancreas was found accidentally during a medical examination. The findings of computed tomography and positron emission tomography-computed tomography suggested a stromal tumor or malignant lymphoma. Surgical resection was performed. The lesions were pathologically diagnosed as duodenal pseudolymphoma.

Background: Cutaneous lymphoid hyperplasia (CLH) is generally classified according to clinicopathologic entities or put into broad spectrums of B-cell or T-cell predominance or co-dominance., Objective: We sought to discern histologic features and immunohistochemical staining patterns in CLH that may form a basis for a histologic classification system., Methods: We studied the clinical, histologic, immunophenotypical, and molecular characteristics of 24 consecutive patients with CLH., Results: The 24 cases were classified according to characteristic histologic features and immunophenotypical staining patterns as follows: presence of germinal center (GC) cell clusters forming well-defined lymphoid follicles (n = 10); presence of clusters of GC cell clusters not forming well-defined lymphoid follicles (n = 6); persistent arthropod assault type CLH (n = 1); CLH with a prominent histiocytic component (n = 4); and CLH without specific histologic and immunophenotypical features, that is, nonspecific mixed T-cell and B-cell CLH (n = 3). Most of the CLH cases did not demonstrate clonal T-cell receptor and/or immunoglobulin heavy chain gene rearrangements except for 3 cases in which the long-term follow-up was uneventful., Limitations: There were a limited number of cases in our study., Conclusions: A classification based on characteristic histologic features and immunophenotypical staining patterns, along with pertinent clinical and molecular data, may enhance the diagnosis of CLH., (Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Reactive lymphoid hyperplasia (RLH) is a benign non-specific lesion having an unknown etiology and pathogenesis. The lesion is found in various organs but is rare in the liver. We report 3 cases of hepatic RLH associated with an extrahepatic malignant tumor. We also provide a literature review based on a search of the PubMed database from 1983 to 2009. The 3 cases showed radiological findings similar to those for malignant tumors and all cases were misdiagnosed as malignant tumors on the basis of these findings. It is difficult to distinguish RLH from malignant or metastatic tumors on the basis of imaging findings. Hepatic RLH is a rare entity and may cause a false diagnosis of malignancy. Because RLH occurs most commonly in middle-aged females, diagnosis of a hypervascular tumor of the liver requires particular care in these patients, especially if an extrahepatic malignancy is present.

Non-random karyotypic abnormalities associated with non-Hodgkin lymphomas (NHLs) have been described in cases of reactive lymphoid hyperplasia (RLH). However, the frequency and types of cytogenetic aberrations detected and their clinical relevance are unknown. To address these questions, we undertook a retrospective analysis of a large series of RLH diagnosed at our institute over 8 years. Cytogenetic abnormalities were identified in 20 of 116 (17%) cases with informative karyotypes, comprising 14 (70%) structural and 11 (55%) numerical changes. Clonal (n  =  14, 70%) and non-clonal (n  =  6, 30%) abnormalities were observed. Aberrations of chromosome 14 were the most frequent (n = 8, 42%, 7 represented IgH translocations), followed by chromosome 3 (n  =  4, 3 represented BCL6 translocations), and chromosome 12 (n = 4). Abnormal karyotypes were most often associated with florid follicular hyperplasia. Isolated lymphoid organ (lymph node, tonsil or spleen) enlargement (12/20, 60%) was more common, no specific etiology was identified in 10/20 (50%) cases and only 1 of 18 patients with clinical follow-up (range 2-107 months, median 60 months) developed lymphoma. In our experience, cytogenetic abnormalities involving loci associated with B-cell NHL are not infrequently detected in RLH. Their occurrence portends low risk for lymphomagenesis, however longer follow-up is prudent to further evaluate the natural history of such cases., (Copyright © 2010 John Wiley & Sons, Ltd.)

Background: We describe a series of previously unreported, distinctive, polypoid solitary T-cell-rich cutaneous pseudolymphomas., Methods: The clinicopathologic features were examined in 17 cases., Results: Patient ages ranged from 16 to 71 years (mean = 38.5) with a female predominance (female : male = 14 : 3). All lesions, clinically diagnosed most often as pyogenic granuloma, presented as a solitary, polypoid, erythematous, papule ranging in size from 2.5 to 7.5 mm (mean = 5.8). Most occurred on the head and neck (7) and trunk (6) with other sites including the thigh (1), shoulder (1) and knee (1). A dense dermal infiltrate composed of mildly atypical lymphocytes with variable numbers of admixed plasma cells and histiocytes was prototypical. Commonly, there was an associated epidermal collarette (16/17), Grenz zone (11/17) or admixed eosinophils (8/17). Prominent vessels lined by plump endothelial cells, reminiscent of high endothelial venules of lymph nodes, were universal and some degree of telangiectasia was also common (12/17). CD3-positive T-cells consisted of an admixture of CD4-positive and CD8-positive forms (15/16). Multiple studies suggested polyclonality (seven cases). No recurrences after lesional excision were noted in the 17 patients with a follow-up range from 24 to 120 months (mean = 46.6)., Conclusion: Although these lesions share histopathologic features of the so-called acral pseudolymphomatous angiokeratoma of children (APACHE), they occur in a completely different clinical setting, present in solitary and polypoid fashion and are T-cell rich. We propose the diagnostic label T-cell-rich angiomatoid polypoid pseudolymphoma for this distinctive but presumably reactive lesion., (Copyright © 2011 John Wiley & Sons A/S.)

B-cell lymphoproliferative disorders are a continuum from benign cutaneous lymphoid hyperplasia (CLH) or "pseudolymphoma" to primary cutaneous B-cell lymphoma (PCBCL). Historically, CLH was treated with a combination of antibiotics, topical or intralesional corticosteroids, and/or localized radiotherapy. Rituximab, a monoclonal antibody that targets the CD20 marker on B cells, is an effective and well-reported treatment for PCBCL. We review the pathogenesis and current treatments of B-cell lymphoproliferative disorders and assess the role of rituximab for potential therapy in the setting of refractory CLH. We describe a case of CLH that was treated with intralesional rituximab. The patient had notable clinical improvement over the treatment period with rituximab. Because of some persistent and recurrent erythematous areas, topical tacrolimus was initiated, with significant clinical improvement. There were no reported side effects. Management of CLH with intralesional rituximab has been described. The treatment presented in this report substantiates rituximab as a reasonable therapeutic option for refractory CLH after failure of several other widely accepted treatments. Treatment with intralesional rituximab should be reserved for patients with documented CD20(+) lesions., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We investigated age-related EBV(+) B-cell lymphoproliferations in the Western population. The clinical features, histology, immunophenotype, EBV-encoded RNA in situ hybridization, and clonality by PCR of T-cell receptor gamma and immunoglobulin genes were categorized in 122 EBV(+) lesions as follows: (1) reactive lymphoid hyperplasia; (2) polymorphic extranodal or (3) polymorphic nodal lymphoproliferative disease (LPD); and (4) diffuse large B-cell lymphoma (DLBCL). Interphase FISH for IG and PAX5 gene rearrangements was performed on 17 cases of DLBCL. The overall median age was 75 years (range, 45-101 years; 67 men, 55 women), and 67, 79, 73, and 77 years, respectively, for groups 1 through 4. Sixteen of 21 cases of polymorphic extranodal LPD were classified as EBV(+) mucocutaneous ulcer. PCR for immunoglobulin genes was polyclonal in reactive lymphoid hyperplasia (84%) and monoclonal in 33%, 63%, and 56% of polymorphic extranodal and nodal LPD cases and DLBCL, respectively. All groups showed restricted/clonal T-cell receptor responses (27%-70%). By FISH, 19% of DLBCLs showed IGH@ rearrangements, but PAX5 was unaffected. Disease-specific 5-year survival was 100%, 93%, 57%, and 25% for groups 1-4, respectively, and 100% for patients with EBV(+) mucocutaneous ulcer. Disease volume was predictive of therapy response (P = .0002), and pathologic subtype was predictive of overall outcome (P = .001). Age-related EBV(+) B-cell LPD encompasses a wider disease spectrum than previously recognized and includes both reactive and neoplastic conditions. Reduction in the T-cell repertoire may contribute to decreased immune surveillance.

Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens., Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining., Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils., Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

Lymphocytoma cutis, also known as cutaneous B cell pseudolymphoma, represents a spectrum of disease that shares similar clinical and histological features and simulates cutaneous lymphoma clinically and histologically. Clinically it is manifested as asymptomatic, indolent, nodular lesions of different sizes varying between 2 and 5 cm, usually solitary, mainly on exposed area of the body like face and neck. The presence of polymorphous cell infiltrates comprising of T and B lymphocytes, plasma cells, oeosinophils, histiocytes and dendritic cells along with lack of atypical lymphocytes after incisional biopsy support diagnosis of pseudolymphoma. Final diagnosis is made on immunohistochemistry.

Conjunctival dermoid cysts are a rare subtype of dermoid cyst usually presenting in adulthood. The authors report a case of a recurrent conjunctival dermoid cyst with reactive lymphoid hyperplasia that was immunohistochemically demonstrated to be polyclonal. Follicular centers that were present within the cyst wall were negative for Bcl-2 and positive for the immunohistochemical markers CD20, Bcl-6, CD10, and Ki67, which helped to differentiate the lesion from a follicular lymphoma. The lesion did not recur after 14 months of follow-up.

Context: Cap-mediated messenger RNA translation controlled by the eukaryotic initiation factor 4F (eIF-4F) complex plays a key role in human cancer. eIF-4F activity is controlled by a repressor binding protein (4E-BP1), which promotes translation when phosphorylated., Objective: To examine the level of expression and phosphorylation of 4E-BP1 in various subtypes of B-cell lymphoma and reactive lymphoid tissues., Design: Archival formalin-fixed, paraffin-embedded B-cell lymphoma samples and reactive lymphoid tissues were immunostained and examined for expression of 4E-BP1 and phosphorylated 4E-BP1. Expression of components of the eIF-4F complex and unphosphorylated and phosphorylated 4E-BP1 was confirmed using Western immunoblotting on lysates of frozen lymphoma samples and reactive tissues., Results: Immunohistochemical analysis demonstrated weak to undetectable 4E-BP1 staining within benign, reactive germinal centers (N = 10). In contrast, 4E-BP1 was consistently expressed (moderate to strong staining) in 98% of various subtypes of mature B-cell lymphoma (N = 50). 4E-BP1 expression was also demonstrable in all 4 lymph nodes with in situ or partial involvement by follicular lymphoma and in all 12 cases of BCL2-negative lymphoma. The level of phosphorylation of 4E-BP1 in lymphomas, evaluated by immunohistochemistry, was heterogeneous., Conclusions: The immunohistochemical expression pattern of 4E-BP1 exhibits regional and cellular specificity in reactive lymphoid tissues and may offer a diagnostic tool for distinguishing reactive follicles from neoplastic B-cell proliferations.