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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nodular lymphocyte predominance Hodgkin's disease (NLPHD), previously called nodular paragranuloma, is a rare entity recognized as a clinico-pathological entity distinct from classic Hodgkin's lymphoma. It is an indolent B cell lymphoma derived from a germinal center cell. NLPHD may closely resemble lymphocyte-rich classic Hodgkin's disease (LR-CHD) or T-cell or histiocyte-rich large B-cell lymphoma (TCRLBCL). A reproducible distinction between these entities is difficult but the classification is prognostically relevant. NLPHD is characterized by neoplastic "popcorn" cells CD20+ CD30- CD15- EMA+ Bcl6+ scattered within a nodular background predominantly composed of small B lymphocytes. LR-CHD neoplastic proliferation is composed of CD20+/- CD30+ CD15+/- EMA- Bcl6+/- Reed Sternberg or Hodgkin's cells, scattered within numerous CD3+ T cells. TCRLBCL is an agressive lymphoma composed of CD20+ CD30- CD15- EMA+/- Bcl6+/- polymorphic neoplastic cells, scattered within a mixture of CD3+ T cells and histiocytes. Epstein Barr virus is detectable within half cases of LR-CHD, but never in NLPHD and rarely in TCRLBCL. The transcription factors BOB1, PU-1, BSAP and IRF4 are new markers that could be useful for differential diagnosis.
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PMID:[Nodular lymphocyte predominance Hodgkin's disease and its differential diagnosis]. 1522 Aug 30

We describe two elderly patients with follicular lymphoma (FL) involving the skin and superficial soft tissues, with a striking proliferation of follicular dendritic cells (FDC). In addition, one patient had bone marrow involvement by FL. Histopathologically, the most remarkable feature in both cases seen at low magnification was a striking pallor of the constituent cells, which were arranged in fascicles, whorls, and round islands. The majority of the cells had the typical cytologic features of FDCs. They were intimately intermingled with centroblasts and centrocytes. A large amount of the clear cytoplasm and the pale nuclei of FDCs, which predominated in the tumors, caused the striking overall pallor of the lesions. Small reactive lymphocytes were scattered between the fascicles. A vague follicular growth pattern was seen only focally. The mantle zones were markedly reduced or absent so that the follicles were seen lying unseparated. The close intermixture of the FDCs and the germinal center cells was responsible for the FDCs appearing to be decorated with B-associated marker, and the germinal center cells seemed to be stained to some degree with FDC-markers. The tumor bulk demonstrated a diffuse and strong reaction with CD10, CD20, CD21, CD35, and stained weakly with CD79a. Fascin and CD23 showed only a weak and focal staining pattern. Bcl-2 decorated large centroblasts and small reactive T-cells. The tumor bulk was negative for actin, EMA, cytokeratins, vimentin, desmin, and factor XIIIa. The proliferative index was rather low; MIB-1 mainly decorated large centroblasts. No monoclonal rearrangement of IgH genes was detected. Epstein-Barr virus was not identified. Electron microscopy revealed typical features of FDCs intermingled with germinal center cells. Such cases may represent a diagnostic pitfall, as FDC overgrowth can mask FL and give the neoplasm the appearance of FDC sarcoma/tumor. We believe that, in both cases, the FDC proliferation had a reactive character.
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PMID:Follicular lymphoma of the skin and superficial soft tissues associated with a prominent follicular dendritic cell proliferation: an unusual pattern which may represent a diagnostic pitfall. 1546 4

Gastric lymphoepithelioma--like carcinoma, undifferentiated with lymphoid or medullary stroma, constitutes a 3.8% of gastric carcinomas. Microscopically it is similar to other lymphoepitheliomas, it has an expansive growth and better prognosis than other histologic types. A possible relation to Epstein Barr virus is presumed. A 56 year old female, gastrectomized due to a cancer (Billroth II) 31 years before, showed an ulcer lesion close to the anastomosis at endoscopy. A biopsy was taken with positive result and gastrectomy was performed. The surgical specimen included gastric stump, jejunoanastomosis, omentum and lymph nodes. It had a 4.5 cm, protruded ulcerated lesion, located in anterior wall, 1.5 cm from stomy. It was fixed in buffered formalin, routinely processed and stained with Hematoxilin--Eosin. Immunohistochemistry techniques for CKAE1-AE3, EMA, CEA, CD45, CD20, CD3 and CD45Ro, and PCR for Epstein Barr virus were performed. Microscopically it was constituted by an expansive proliferation of polygonal cells with barely eosinophylic cytoplasm and vesicular nuclei, arranged in small clusters or isolated, positive for CKAE1-AE3, EMA and CEA, with dense mature lymphoid infiltrate, both follicular and diffuse, positive for CD45, CD45Ro, CD3 and CD20. PCR technique for Epstein Barr virus was positive. This has been the first case of this type in our department since 1989. In addition to the peculiar characteristics of this variety, whose differential diagnosis must be made with lymphomas, the origin from gastric stump, where this complication appears with variable rates, the risk being related to the type of surgery and the time elapsed are relevant.
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PMID:[Lymphoepithelioma-like gastric carcinoma: unusual variant in postgastrectomy stump]. 1548 94

Anaplastic large cell lymphoma (ALCL), CD30+, is a subtype of T-non-Hodgkin's lymphoma (NHL). Its most common form is a classical systemic type that involves multiple nodal and extranodal sites. In this study, morphologic, immunohistologic, and genetic studies were performed on ALCL cases in Pakistani patients. The median age of the patients in this study was 45 years (age range: 5-70 years), with a male to female ratio of 3.4:1. Thirty-seven (37) patients were diagnosed to have Ki-1 (CD30+) ALCL, which constituted 2% of all NHLs and 12.6% of all T-NHLs, over a period of 11 years (January 01, 1992-December 31, 2002). The tumors were of either T- or null-cell type with constant (100%) expression of CD30 (Ki-1). The majority of the cases (89.2%) expressed EMA, whereas 40.5% of the cases expressed either CD45 (LCA), CD45RO (UCHL1), or ALK. The mean age of ALCL patients with null-cell phenotype was 33.8 years as compared to those with T-cell phenotype having a mean age of 36.3 years. Out of the 37 cases diagnosed as ALCL, amplifiable DNA was isolated from 28 cases, which were further assessed for T-cell clonality for T-cell receptor (TCR)-beta, gamma, and immunoglobulin heavy chain (IgH) for the FR2 and FR3 regions. The polymerase chain reaction (PCR) technique demonstrated clonal rearrangement of the TCR beta, gamma, and IgH regions in 15 (53.6%), 11 (39.3%), and 2 (7.1%) ALCL cases, respectively, out of 28 cases. Association of Epstein-Barr virus (EBV) was noted in seven out of 28 cases (25%) of ALCL by PCR, whereas ISH for EBV-encoded nuclear RNA-1 (EBER-1) detected the presence of EBV in two (16.7%) out of 12 cases, where one was T-cell ALCL and the other null-cell ALCL. Immunostaining for LMP-1 could not be performed, because tissue material was not available. In conclusion, our study demonstrated that the prevalence of ALCL in Pakistan is comparable to that reported for some of the Asian communities and by the International Lymphoma Study Group and that EBV could be partly responsible for the pathogenesis of ALCL.
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PMID:Prevalence and characterization of anaplastic large cell lymphoma and its association with Epstein-Barr virus in Pakistani patients. 1564 4

Plasmablastic lymphoma is an HIV-associated non-Hodgkin's lymphoma that primarily affects the oral cavity and jaws. The purpose of this report is to describe the first case of plasmablastic lymphoma occurring in an HIV-negative, nonimmunocompromised individual, and to review the histopathologic and immunohistochemical phenotype of this lymphoma. Histopathologically, our case exhibited a dense, diffuse lymphocytic infiltrate of noncohesive large lymphocytes with plasmacytoid features. Immunohistochemical analysis revealed positivity for the B-cell marker CD79a, VS38c, Epstein-Barr virus latent membrane protein (LMP), immunoglobulin G (IgG), and lambda light chain restriction. Neoplastic cells were negative for leukocyte common antigen, CD20, CD3, CD10, CD138, Bcl-2, Bcl-6, desmin, actin, EMA, S-100, HMB45, Alk-1, HHV8, IgA, IgM, and cytokeratin. The features of this rare disease are summarized based on a comprehensive review of the epidemiologic, clinical and immunohistochemical findings of previously reported cases.
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PMID:Oral plasmablastic lymphoma in an HIV-negative patient: a case report and review of the literature. 1603 78

The lymphohistiocytoid variant of diffuse malignant mesothelioma is rare with very few cases described in the literature. It is characterized by mesothelial cells with a histiocytelike appearance and an associated dense lymphoid infiltrate. We studied clinicopathologic features and immunohistochemical patterns of a series of 22 cases. The histiocytelike cells had a mesothelial immunophenotype: AE1/AE3 (100%), calretinin (100%), CK5/6 (46%), and EMA (52%). The prominent lymphoid component showed a cytotoxic T-cell immunophenotype. Prognosis was similar to that of a large series of epithelioid diffuse malignant mesotheliomas. Formely, it was classified within the sarcomatoid type. We suggest that it should be reclassified as an epithelioid variant because of its similar behavioural characteristics. There was no evidence of Epstein-Barr virus-related infection.
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PMID:Lymphohistiocytoid variant of malignant mesothelioma of the pleura: a series of 22 cases. 1746 Apr 54

The term lymphoepithelioma-like carcinoma identifies a group of nasopharingeal epithelial tumors characterized by aggregates of malignant undifferentiated cells surrounded by a dense reactive lymphoplasmacellular infiltrate. Primary cutaneous localization is rare, with approximately 30 cases reported in literature. We describe a case of primary lymphoepithelioma-like carcinoma of the skin in a 92-year-old woman. Immunohistochemical examination was positive for cytokeratine (KL1 and EMA) as regards epithelial cells, while the lymphocitic infiltrate was positive for LCA and CD3. In situ hybridization for Epstein Barr virus in tumor cells was negative. Electron microscopy showed rounded and occasionally spindle-shaped poorly-differentiated squamous epithelial cells, and a lymphoid stroma consisting mostly of normal-appearing small lymphocytes. Examination of the nasopharynx did not show any tumoral mass and after a 7 years follow-up the patient is free of local and distant recurrences. This tumor affects people aged over 50 years and is localized to the face, but scalp, shoulder and forearm may be involved. Research of Epstein-Barr virus is always negative in this tumor, unlike nasopharingeal epithelial carcinoma. The differential diagnosis of lymphoepithelioma-like carcinoma of the skin may present some difficulties and includes squamous cell carcinoma. Lymphoepithelioma-like carcinoma of the skin is a malignant neoplasm which tends to relapse locally and has a moderate tendency to metastatize.
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PMID:Lymphoepithelioma-like carcinoma of the skin. 1817 60

A case of CD30-positive microvillous lymphoma (MVL) in an 87-year-old man who was encountered generalised lymphadenopathy is presented. Histopathologically, the tumour showed a morphological mimic of anaplastic large cell lymphoma (ALCL) with sinusoidal growth pattern. Immunohistochemically (IHC), the tumour cells were CD30(+), CD20(+), CD45(+), BCL-2(+), BCL-6(+), MUM1(+), Ki-67(+), CD45RO(-), CD3(-), CD10(-), CD15(-), CD56(-), EMA(-), TIA-1(-) and ALK(-). Flow cytometry confirmed the IHC. In situ hybridisation for Epstein-Barr virus RNA was negative. Electron microscopically, the tumour cells were similar to large transformed lymphocytes and had circumferentially profuse microvillous projections resembling those of epithelial mesothelioma cells. In conclusion, CD30-positive MVLs are indistinguishable from ALCLs that have ultrastructural microvillous projections by morphology alone. However, the lack of EMA, TIA-1 and ALK expression in this MVL case facilitated a definite distinction from ALCLs. The results of a panel of three markers (CD10(-), Bcl-6(+) and MUM1(+)) suggested that the present case of CD30-positive MVLs has an activated non-germinal centre B-cell origin.
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PMID:CD30-positive diffuse large B-cell lymphoma with microvillous features: so-called microvillous lymphoma. 1912 65

Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.
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PMID:Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia. 1948 35

Hodgkin lymphoma (HL) is a malignancy of the lymph system usually occurring on the lymph nodes. A 57-year-old Japanese woman presented with the chief complaint of an enlarging tumor of the left ear. An excisional biopsy was taken, and histological examination showed a mixed infiltration of cells, including Reed-Sternberg cells and their mononuclear forms against a background of small lymphocytes. Reed-Sternberg cells were CD15, CD20, CD30, Ki-67, MUM-1, CD45, EMA, and Epstein-Barr virus-encoded small RNA was detected by in situ hybridization. We diagnosed this tumor as a skin infiltration with a lymphocyte-rich classical HL pattern. Skin involvement of HL is most often a secondary phenomenon representing a rare late manifestation of disease dissemination; however, we could not detect any evidence of systemic lesion for 6 months after the initial presentation. A case of HL only involving the skin was reported by several past reports, which termed it primary cutaneous HL. But, it is still controversial whether HL initially occurs on the skin because a diagnostic gray zone exists between HL, some non-HL entities, and nonneoplastic lymphoid infiltrates. Clinical and histological features of this case suggest that the skin will become a primary site of HL.
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PMID:Epstein-Barr virus-positive Hodgkin lymphoma-like earlobe lymphoid infiltrate: case report. 1977 Jun 29


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