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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A biopsy of a nasal mass that had morphologic and immunostaining features consistent with a B-cell lymphoma was studied for clonality using PCR of the IgH gene. An unexpectedly low molecular weight DNA fragment of approximately 140bp (acceptable size limit: 250-295bp) was obtained using FR2 and JH primers. The sequence of this DNA was consistent with a clonal IgH rearrangement followed by a deletion that removed most of the downstream portion of the V segment. Thus, the biopsy contained a monoclonal population of B-lymphocytes, consistent with a diagnosis of lymphoma. This work illustrates that bands outside of the size range expected from PCR of the antigen receptor genes may still be consistent with a monoclonal result.
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PMID:Detection of clonality in lymphoproliferations using PCR of the antigen receptor genes: does size matter? 1770 69

Gene expression profiling of diffuse large B-cell lymphoma (DLBCL) has revealed biologically and prognostically distinct subgroups: germinal center B-cell-like (GCB), activated B-cell-like (ABC) and primary mediastinal (PM) DLBCL. The BCL6 gene is often translocated and/or mutated in DLBCL. Therefore, we examined the BCL6 molecular alterations in these DLBCL subgroups, and their impact on BCL6 expression and BCL6 target gene repression. BCL6 translocations at the major breakpoint region (MBR) were detected in 25 (18.8%) of 133 DLBCL cases, with a higher frequency in the PM (33%) and ABC (24%) subgroups than in the GCB (10%) subgroup. Translocations at the alternative breakpoint region (ABR) were detected in five (6.4%) of 78 DLBCL cases, with three cases in ABC and one case each in the GCB and the unclassifiable subgroups. The translocated cases involved IgH and non-IgH partners in about equal frequency and were not associated with different levels of BCL6 mRNA and protein expression. BCL6 mutations were detected in 61% of DLBCL cases, with a significantly higher frequency in the GCB and PM subgroups (>70%) than in the ABC subgroup (44%). Exon-1 mutations were mostly observed in the GCB subgroup. The repression of known BCL6 target genes correlated with the level of BCL6 mRNA and protein expression in GCB and ABC subgroups but not with BCL6 translocation and intronic mutations. No clear inverse correlation between BCL6 expression and p53 expression was observed. Patients with higher BCL6 mRNA or protein expression had a significantly better overall survival. The biological role of BCL6 in translocated cases where repression of known target genes is not demonstrated is intriguing and warrants further investigation.
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PMID:Distinctive patterns of BCL6 molecular alterations and their functional consequences in different subgroups of diffuse large B-cell lymphoma. 1762 4

Clonal B-cell populations have been described in peripheral T-cell lymphomas (PTCL) as secondary Epstein-Barr virus (EBV) driven B-cell expansions that may evolve to an overt B-cell lymphoma. EBV-negative B-cell proliferations associated with T-cell lymphomas are uncommon and not well characterized. We studied 15 patients who developed an EBV-negative B-cell proliferation or malignant lymphoma associated with PTCL. The T-cell tumors were 8 PTCL, not otherwise specified, 4 angioimmunoblastic T-cell lymphomas, and 3 cutaneous PTCL. The B-cell component was intermingled with the PTCL in all patients and it was classified as clonal/monotypic plasma cell proliferation in 8 lesions, clonal/monotypic large B-cell proliferation in 4 patients, and B-cell lymphoma with plasmacytic/plasmablastic differentiation in 3 patients. Two patients had 2 clonally unrelated plasma cell proliferations associated with the same PTCL. All cases showed cytoplasmic Ig light chain restriction. Clonal IgH and T-cell receptor rearrangements were detected in 11/12 and 11/13 cases examined, respectively. EBV, cytomegalovirus, and HHV-8 were not observed in any of the examined cases. Sequential samples in 7 patients showed persistence of the PTCL and the B-cell component in 4, the PTCL without the B-cell lymphoma in 2, and progression of the B-cell neoplasm in 1. Patients followed an aggressive clinical course similar to conventional PTCL. In conclusion, EBV-negative clonal or mononotypic B-cell proliferations in patients with PTCL present with a spectrum of lesions ranging from plasma cell proliferations to overt lymphomas with plasmacytic/plasmablastic features. The distinctive features of these patients suggest that these lesions represent a specific phenomenon in PTCL.
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PMID:Epstein-Barr virus negative clonal plasma cell proliferations and lymphomas in peripheral T-cell lymphomas: a phenomenon with distinctive clinicopathologic features. 1772 Nov 85

The presence of circulating villous lymphocytes (VLs) in lymphoma patients usually points to splenic marginal zone B-cell lymphoma (SMZL), even if the VLs can be found occasionally in other small B-cell lymphomas. However, those cells are variably described, and detailed cytologic characterization is often lacking. We identified lymphoma cases with numerous basophilic VLs among the large group of splenic lymphoma with VLs, and for further delineation, 37 cases with this particular cytology were analyzed. Patients, predominantly older men, presented with moderate lymphocytosis and splenomegaly without pancytopenia. The monoclonal B cells expressed IgM + D, IgM + G, IgM or IgG, as well as CD76 and CD11c, frequently CD103, and rarely CD123. Spleen sections were peculiar, with atrophic white pulp and a monomorphic diffuse lymphoma infiltration in a congested red pulp. Bone marrow infiltration was interstitial and intrasinusoidal without extensive fibrosis. Cytogenetic analysis showed a frequent absence of clonal aberrations (68%). Most cases (79%) were IgH mutated, with an overrepresentation of V(H)3 and V(H)4 gene families. These results, as well as the clinical evolution, show that those lymphoma cases represent a homogeneous group distinct from SMZL and reminiscent of hairy cell leukemia variant, perhaps corresponding to a separate lymphoma entity.
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PMID:Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity? 1804 95

Castleman's disease (CD) of the pancreas/peripancreas is extremely rare. The recently introduced, endoscopic ultrasonography (EUS)-guided trucut biopsy (TCB) is a useful diagnostic modality for obtaining tissue samples from peripancreatic lesions. However, its role in diagnosing CD remains unknown. We report a case of localized, peripancreatic, hyaline-vascular CD biopsied using EUS. The pathology results were initially interpreted as an extranodal, marginal-zone B-cell lymphoma. However, polymerase chain reaction (PCR) study for the IgH gene rearrangement revealed a polyclonal pattern. We also reviewed the relevant literature. To our knowledge, this is the first illustrated report on EUS-TCB findings of CD with its pathology results of EUS-TCB mimicked a B-cell lymphoma.
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PMID:Endoscopic ultrasonography-guided trucut biopsy for the preoperative diagnosis of peripancreatic Castleman's disease: a case report. 1839 17

The characteristics of de novo diffuse large B-cell lymphoma (DLBCL) with translocation of c-myc and immunoglobulin (Ig) genes (c-myc/Ig DLBCL), were investigated in 13 cases of c-myc/Ig DLBCL. Immunohistochemistry revealed five cases were positive for CD10 and BCL6 expression (CD10(+)/BCL6(+)), five cases of CD10(-)/BCL6(+)/MUM1(-), one case of CD10(-)/BCL6(+)/MUM1(+) and two cases of CD10(-)/BCL6(-)/MUM1(+) expression, indicating 10 cases of germinal center B-cell DLBCL and three cases of non-germinal center B-cell DLBCL. Ongoing mutation of the Ig heavy chain gene variable region (IgH-V) was found in two cases with CD10 and BCL6 expression and one case showing CD10(-)/BCL6(+)/MUM1(-) expression. These three cases of ongoing mutation of the IgH-V gene did not express BCL2, unlike those without ongoing mutation. These results suggest a heterogeneous immunophenotype and genotype for c-myc/Ig DLBCL, with CD10(-)/BCL6(+)/MUM1(-) cases the most frequent.
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PMID:Characterization of de novo diffuse large B-cell lymphoma with a translocation of c-myc and immunoglobulin genes. 1846 Apr 3

Lymphoplasmacytic lymphoma (LPL) is a small B-cell lymphoma with plasmacytic differentiation that does not fulfill the criteria for any other small B-cell lymphoma. Cytogenetic characterization of nodal LPL is limited and the distinction from marginal zone lymphomas with plasmacytic differentiation can be problematic. Thus, 17 cases of lymph node-based LPL were studied with fluorescence immunophenotypic and interphase cytogenetics for the investigation of neoplasia (FICTION) using a CD79a antibody and probes to detect trisomies of chromosomes 3 (15 cases), 12 (16 cases), and 18 (17 cases); rearrangements (R) of IgH (10 cases), BCL6 (6 cases), PAX5 (7 cases), and MALT1 (16 cases); and deletion 6q21 (7 cases). Cases with IgH R were further studied with an IgH/BCL2 probe. In cases without FICTION studies, previously reported fluorescence in situ hybridization results for IgH, PAX5, and deletion 6q21 were available from prior studies. The histopathology, immunophenotype, and available clinical data were also reviewed. Three pathologic categories were recognized: 5 classic LPL, 5 vaguely nodular polymorphous (VN-P), and 7 other. Among the classic LPL, 4/4 had an IgM paraproteinemia, 5/5 had bone marrow involvement (BM+), and 1/5 had +MALT1. One of one VN-P LPL had an IgM paraprotein, 2/4 were IgM+, 2/4 IgG+, 1/3 had BM+, and 1/5 had an IgH R. Among the other cases, 2/3 had a paraprotein, 2/7 were IgM+, 5/7 IgG+, and 0/3 had BM+. Of these cases, 1 showed +12, 1 +18, and 1 IgH/BCL2 rearrangement plus +18. None of the 17 cases had a 6q21 deletion or +3. Therefore, with rare exception, lymph node-based LPL with classic or more varied histopathologic features does not have the cytogenetic abnormalities frequently associated with bone marrow-based LPL/Waldenstrom macroglobulinemia or many of the marginal zone lymphomas. The search for better objective inclusionary criteria for LPL must continue.
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PMID:Fluorescence immunophenotypic and interphase cytogenetic characterization of nodal lymphoplasmacytic lymphoma. 1867 Mar 52

We studied 53 cutaneous lymphoproliferative disorders, all of which manifested hair follicle hyperplasia. There were 42 cases conforming to the description of pseudolymphomatous folliculitis (PLF) and 11 cases of authentic lymphomas including mycosis fungoides, CD30+ anaplastic large cell lymphoma, diffuse large B-cell lymphoma, B-cell small cell lymphoma/leukemia, and peripheral T-cell lymphoma, not otherwise specified. All patients with PLF clinically presented with a solitary nodule preferentially involving the face. Beside hair follicle hyperplasia, the typical features were a dense infiltrate of small well-differentiated lymphocytes, lymphoplasmacytoid cells, plasma cells, and epithelioid histiocytes forming tiny granulomas. Some unusual or worrisome features recognized included eccrine/apocrine duct hyperplasia, subcutis/muscle infiltration, lymphocyte "smudging," single file infiltration, and large atypical cells. Immunohistochemically, T-cell predominant cases dominated in the series. All 34 tested cases revealed a polyclonal pattern of kappa and lambda immunoglobulin (Ig) light chain expression. In 4 cases, scattered CD30+ cells were identified. Monoclonal rearrangements of T-cell receptor (TCR) and IgH genes were detected in 19 and 3 cases respectively, including 1 case with dual T-cell receptor/IgH rearrangement. Three of 30 tested cases proved positive for herpes simplex virus-1, whereas herpes simplex virus-2 always tested negative. Of 31 cases tested for Borrelia burgdorferi, 30 specimens were negative. In 9 cases, fluorescent in situ hybridization for t(11;18) and t(14;18) revealed none of the above translocations. The most common treatment modality was surgical removal. Forty patients with a mean follow-up of 3.7 years included 39 patients with no evidence of disease and 1 individual with local recurrence. The comparison of "clonal cases of PLF" and those with polyclonal population or in which clonality remained undetermined revealed no differences between the 2 groups in the clinical presentation, pathologic, and immunohistochemical features. We conclude that hyperplasia of hair follicles and other adnexa can be seen not only in the condition currently known as PLF, but also in genuine cutaneous lymphomas and may be just a happenstance secondary to a basic pathologic process.
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PMID:Hyperplasia of hair follicles and other adnexal structures in cutaneous lymphoproliferative disorders: a study of 53 cases, including so-called pseudolymphomatous folliculitis and overt lymphomas. 1868 86

We report the clinical and immunohistological features of two cases of chronic lymphocytic leukaemia (CLL) with Hodgkin's transformation. These cases occurred in a 70-year-old man with a three-year history of CLL and in a 76-year-old man with a few months history of CLL. Microscopic examination showed the presence of large tumor cells with the morphological and immunophenotypic features of classical Hodgkin and Reed-Sternberg (R-S) cells, in a background of otherwise typical B-CLL. The transformation of CLL into large B cell lymphoma (Richter's syndrome) is a well-documented phenomenon. Only rarely does CLL transform into Hodgkin's lymphoma, but this diagnosis is often easy and offers few differential diagnoses. The major points of interest lie in the pathogenetic relationship between CLL and Hodgkin's disease, and in the potential clinical implications of this peculiar condition. Literature on the subject indicates that identical IgH gene rearrangements in micromanipulated R-S and CLL cells have been identified in 7/12 cases. In these patients, the R-S and CLL cells belong to the same clonal population, suggesting a progression from the underlying CLL cells. This group appears to have a poor prognosis, identical to classical Richter's syndrome. In other cases, the R-S cells were often Epstein-Barr virus (EBV) positive and did not share the clonal rearrangements identified in CLL cells, suggesting that Hodgkin's disease in these patients could represent a second malignancy, EBV-related and favored by immunosuppression, associated with a better prognosis.
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PMID:[Hodgkin's disease variant of Richter's syndrome. Two cases and literature review]. 1892 73

A 49-year-old HIV-positive Japanese man was referred to our hospital for multiple skin nodules. Many plasmablastic atypical lymphocytes were observed in the peripheral blood. He was diagnosed with diffuse large B cell lymphoma (DLBCL) by a biopsy of the inguinal lymph node. IgH/MYC translocation was detected by in situ hybridization of the lymph node and chromosomal analysis of bone marrow cells showed 46, XY, t(8 ; 14)(q24 ; q32)add(14)(q32), der(21)t(1 ; 21)(q12 ; p11). He showed a transient response to multi-agent chemotherapy, and during the course of salvage chemotherapy, he died of urinary infection. This case has unique clinical features compared with previously reported DLBCLs with plasmablastic differentiation.
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PMID:CD20-negative CD138-positive leukemic large cell lymphoma with plasmablastic differentiation with an IgH/MYC translocation in an HIV-positive patient. 1933 59


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