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Query: UNIPROT:Q06643 (non-Hodgkin's lymphoma)
11,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary effusion lymphoma (PEL) represents a novel B-cell non-Hodgkin's lymphoma (NHL) type associated with Kaposi's sarcoma-associated herpesvirus infection and typically growing as lymphomatous effusions in the body cavities. The precise B-cell subset from which PEL originates as well as the biologic mechanisms responsible for its peculiar growth pattern are unclear. In this study, we have analyzed PEL for the expression status of CD138/syndecan-1, a molecule selectively associated with late stages of B-cell differentiation and implicated in cell-to-cell and cell-to-extracellular matrix interactions. PEL patient samples (n = 7) and cell lines (n = 5) were investigated by multiple approaches, including immunocytochemistry, flow cytometry, RNA analysis, and Western blot studies. For comparison, lymphomatous effusions other than PEL (n = 13) and tissue-based NHL (n = 103) were also tested. Expression of CD138/syndecan-1 associates at high frequency with PEL (5 of 7 patient samples and 5 of 5 cell lines), whereas it is consistently absent among other lymphomatous effusions (n = 13). The CD138/syndecan-1 isoform expressed by PEL has an average molecular weight of 420 kD, which is substantially different from that of CD138/syndecan-1 molecules generally expressed by plasma cells. These data, along with previous immunophenotypic evidence, unequivocally define that PEL cells represent a preterminal stage of B-cell differentiation and may bear implications for the peculiar growth pattern of this lymphoma.
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PMID:Association of Kaposi's sarcoma-associated herpesvirus-positive primary effusion lymphoma with expression of the CD138/syndecan-1 antigen. 938 6

We describe an HIV-infected 44-year-old man who presented 1 month after discontinuation of HAART therapy with a large mass extending from the mediastinum, enclosing the heart and extending through the diaphragm to the epigastric region. Biopsies subsequently revealed a highly aggressive non-Hodgkin's lymphoma (NHL) producing sheets of cells with an organoid distribution. The cells had abundant basophilic cytoplasm and a plasmacytic appearance. Although immunohistochemistry failed to show either B- or T-cell markers, antigens consistent with plasma cells were found. An immunoglobulin heavy chain clonal rearrangement was identified by PCR analysis. These studies were supportive of a diagnosis of a plasmablastic lymphoma. While awaiting the results of these tests, the patient was reinitiated on his HAART regimen. He was found on follow-up a month later to have complete resolution of his bulky mediastinal mass. He remained free of disease for 3 months with subsequent rectal and abdominal recurrence. Treatment with CHOP chemotherapy with filgrastim support was begun which resulted in another remission. Plasmablastic lymphoma is now reported in some studies to account for 2.6% of all HIV-related NHL. Originally described in 1997 in a series of 16 patients, this entity is highly associated with HIV infection in its later stages. Often, patients present with oral or jaw lesions with a rapidly progressive course. The tumors have the morphologic appearance of a plasmacytoid tumor with high proliferative index. Markers are positive mainly for LCA, CD79a, VS38C, and CD138. Co-infection with HHV-8 and EBV has not been consistently reported. Therapy with standard regimens has variable response. One case has been reported with a 3.5 year disease free survival. The regression of disease after resumption of HAART therapy alone in this patient suggests that HAART has an important role in the treatment of lymphoma in the HIV infected patient.
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PMID:Regression of a plasmablastic lymphoma in a patient with HIV on highly active antiretroviral therapy. 1199 80

Gaucher-like cells have occasionally been described in various haematological malignancies including Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma (MM) and chronic myelogenous leukaemia (CML). A special type of this phenomenon is crystal-storing histocytosis or the so-called pseudo-pseudo Gaucher cells (PPGC) in which crystalline protein storage in macrophages is induced by paraproteinemia. Here we describe a 54-year-old man with an initial suspicion of Gaucher disease and monoclonal IgA gammopathy in whom a correct diagnosis of lymphoplasmacytic lymphoma (LPL) with massive infiltration of bone marrow and spleen by PPGC was confirmed by immunological, ultrastructural and molecular characterisation. The activity of leukocyte beta-glucocerebrosidase was only slightly elevated (7.3 nmol/mg protein/1 h) which ruled out the diagnosis of classic Gaucher's disease. The patient received two courses of CHOP without improvement and anti-CD20 monoclonal antibody (rituximab) with only temporary stabilisation. Subsequently, he underwent splenectomy because of prolonged severe pancytopenia and a suspicion of hypersplenism. After splenectomy significant haematological improvement was observed. Following anti-CD20 therapy, changes in immunoprofile and morphology of tumour cells were evident. Before treatment the population of LPL was more divergent, with expression of LCA, CD20, CD38 and CD138. However, after the treatment, there were more mature plasma cells which no longer expressed CD20 antigen-this picture was more consistent with the diagnosis of plasma cell myeloma. Similarly, in the spleen there were no CD-20-positive cells evident. Finally, the patient received two courses of VAD vincristine, doxorubicin, dexamethasone) with further haematological improvement but complete response was not achieved.
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PMID:Lymphoplasmacytic lymphoma with monoclonal gammopathy-related pseudo-Gaucher cell infiltration in bone marrow and spleen--diagnostic and therapeutic dilemmas. 1261 22

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL), a proliferating peripheral B-cell neoplasm, is a morphologic variant of diffuse large B-cell lymphoma (DLBCL), which may be confused with Hodgkin's lymphoma, non-Hodgkin's lymphoma, and reactive lymphadenopathies. Though more recent studies suggested that it might be a distinct clinicopathologic entity and/or a heterogeneous entity with derivation from germinal center B cells, its histogenetic derivation remains controversial. The authors analyzed 30 cases of THRLBCL to further characterize the origin of the neoplastic cells using immunohistochemical and molecular studies for expression of Bcl-6, CD10, and CD138, as well as rearrangements of IgH/bcl-2 genes on paraffin-embedded tissue. Half of the cases (15/30) showed Bcl-6 expression and five cases (19%) showed CD10 expression, but none had CD138 expression (0/20). Only three cases showed coexpression of both Bcl-6 and CD10. Molecular studies performed in 21 cases detected rearrangement of immunoglobulin heavy gene in 18 cases, with none having detectable Bcl-2 gene rearrangement. These data indicate that similar to DLBCL, the cell origin of neoplastic cells in THRLBCL is composed of a heterogeneous group of proliferating peripheral B cells, with only some cases originating from germinal center B cells and others derived from heterogeneous origins. Lack of Bcl-2 gene rearrangements seems to argue against a possible progression from preexisting follicular lymphoma. Thus, the normal counterpart of the neoplastic cells cannot at this time be the sole basis for the subclassification of THRLBCL.
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PMID:T-cell/histiocyte-rich large B-cell lymphoma displays a heterogeneity similar to diffuse large B-cell lymphoma: a clinicopathologic, immunohistochemical, and molecular study of 30 cases. 1589 21

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

A 7-year-old boy with a positive history of vertical HIV transmission presented with a painful swelling over the left upper jaw of 20 days' duration. A provisional diagnosis of non-Hodgkin's lymphoma or embryonal rhabdomyosarcoma was made. Fine-needle aspiration cytology findings were inconclusive, and incisional biopsy tissue on routine stains gave an impression of small round cell tumor with plasmacytoid features. A series of histochemical stains and immunohistochemical studies was carried out to differentiate and characterize this tumor. The salient immunostaining was negativity with all routine small round cell tumor markers and positivity with CD138 and lambda light chain restriction. The heterogeneous presentation of plasmablastic lymphoma as a variant of diffuse large B-cell lymphoma and its histogenesis is documented. Potential pitfalls and differential characterization of AIDS-plasmablastic lymphoma from other closely related tumors are addressed. The importance of excluding other common pediatric small round cell tumors by immunohistochemistry is highlighted. To the best of our knowledge, this is the first report of this entity in a vertically transmitted HIV-positive child.
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PMID:Plasmablastic lymphoma of the oral cavity in an HIV-positive child. 1630 Nov 54

Primary effusion lymphoma (PEL) is an unusual form of non-Hodgkin's lymphoma, which is characterized by lymphomatous effusion in body cavities, but no associated mass lesions. It is usually associated with an immunodeficient state most often with the human immunodeficiency virus (HIV). We describe a 54-year-old man with HIV-negative PEL, with a history of hepatitis B virus-related liver cirrhosis. Both abdominal and pleural cavities were involved; no solid tumor masses were found and bone marrow investigations were normal. The ascites and pleural effusion contained numerous pleomorphic lymphoid cells. Immunophenotyping was positive for CD138. Chromosome study showed complex cytogenetics. The genomic human herpesvirus-8 was detected in the lymphoma cells. It is postulated that the immunosuppressed state in this patient may have been caused by cirrhosis. The patient received four cycles of chemotherapy of CHOP and Picibanil (OK-432) intraperitoneal administration. However, no durable remission was achieved. Adefovir failed to halt the progressive liver failure after the development of YMDD mutant related to lamivudine. He died of sepsis and hepatic failure.
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PMID:Primary effusion lymphoma involving both pleural and abdominal cavities in a patient with hepatitis B virus-related liver cirrhosis. 1758 46

Plasmablastic lymphoma (PBL) has been recently characterised as an aggressive subtype of non-Hodgkin's lymphoma, most frequently arising in the oral cavity of HIV-infected patients. To date, approximately 60 cases fulfilling the clinico-pathological characteristics of PBL have been reported. PBLs are composed of large cells with eccentrically located nuclei and deeply basophilic cytoplasm with a paranuclear hof. The tumour cells are invariably immunoreactive for the plasma cell marker CD138, and show monoclonal rearrangement of the immunoglobulin heavy chain gene (IgH) and/or clonal restriction of the Ig light chain (IgL) gene expression in most of the cases. Similar to other types of AIDS-related lymphomas, there is evidence that Epstein-Barr virus and Kaposi-sarcoma associated Human Herpes Virus 8 may play a relevant role in the pathogenesis of PBL. PBL patients have been treated heterogeneously, with a combination of chemotherapy, radiotherapy and/or surgery, and their prognosis is usually poor, with a death rate of approximately 60% at 1 year.
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PMID:Plasmablastic lymphoma: a review. 1893 60

Primary effusion lymphoma (PEL) is an unusual and rare type of non-Hodgkin's lymphoma characterized by lymphomatous effusion of pleural, pericardial or peritoneal cavities without lymphadenopathy or organomegaly. It is associated with human herpes virus-8 (HHV-8) and occurs most often in immunodeficient patients. We present a case of PEL in a 69-year-old male presenting with pleural effusion and ascites. Fluid aspiration showed a monomorphic population of atypical lymphoid cells, which were medium- to large-sized, with mono- or binucleated hyperchromatic nuclei and a small to moderate amount of basophilic cytoplasm containing cytoplasmic vesicles. Immunohistochemically, the lymphoid cells expressed CD138 and multiple myeloma oncogene 1, were positive for HHV-8, and were monoclonal for immunoglobulin heavy chain gene rearrangement. They were negative for Epstein-Barr virus by in situ hybridization. Unfortunately, the patient died during the first course of chemotherapy with cyclophosphamide, vincristine and prednisone.
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PMID:Cytologic diagnosis of primary effusion lymphoma in an HIV-negative patient. 1918 87

Primary effusion lymphoma (PEL) is very rare type of non-Hodgkin's lymphoma (NHL) usually confined to the body cavities such as the pleural space, pericardium, and peritoneum. PEL is a human herpes virus-8 (HHV-8)-associated lymphoma and commonly observed in human immunodeficiency virus (HIV)-infected patients. However, HIV-infected patients are extremely fewer in Japan in comparison with those in Western countries; PEL is usually not associated with HIV infection in Japan. This report presents seven Japanese cases of PEL. In situ hybridization revealed that the PEL cells were negative for EBV in all cases. An immunocytological analysis showed that only one case was positive for HHV-8, and PEL cells were positive for CD20 in all cases. MUM1 was positive, but CD10 and CD138 were negative in six cases. One case each was positive for CD30 and BCL-6. The phenotypic patterns of HIV-related is BCL6-/MUM1+/CD138+, thus, the phenotypic findings observed by immunocytochemistry in this study were somehow different from those reported in Western countries. However, the cytomorphological features of PEL cells showed large cell size, abundant basophilic cytoplasm, coarse chromatin, and occasional binucleated or multinucleated cells, similar to a large cell immunoblastic and anaplastic large cell lymphoma, indicating that the cytomorphological characteristics of PE cells in Giemsa and Papanicolaou stain were consistent with those reported abroad. The prognosis for PEL in these cases was poor, but the survival time was variable ranging from 1 month to 54 months, and was different from that of Western cases. No p16/CDKN2A expression was observed, and one case showed PEL cells with a BLIMP1 mutation.
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PMID:Cytologic and immunocytochemical features of EBV negative primary effusion lymphoma: report on seven Japanese cases. 1921 41


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