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

These are the first cases of primary macroglobulinemia (PMG) with t(11;18)(q21;q21) reported in the literature. The first case was a 77-year-old man with macroglobulinemia (serum IgM: 8.36 g/dL). Abnormal lymphoid cells were detected in the blood and bone marrow. Immunologic and karyotypic analyses revealed that abnormal cells were positive for surface IgM-k, CD19, and CD20, negative for CD5 and CD10, and all had a t(11;18)(q21;q21). The second case was a 57-year-old woman with macroglobulinemia (serum IgM: 12.0 g/dL). Abnormal lymphoid cells were detected in blood and marrow, and cells were positive for surface IgM-lambda, CD19, and CD20, and negative for CD5 and CD10. Plasma cells bearing cytoplasmic IgM-lambda were increased in pleural fluid. Karyotyping demonstrated t(2;11;18)(q21-23;q21;q21). Rearrangements within BCL2 and YES genes located at 18q21 were not detected. Sixteen other cases with t(11;18)(q21;q21) have been reported in marginal zone B-cell lymphoma. Therefore, our report is in agreement with the finding that part of primary macroglobulinemia is a variant of marginal zone B-cell lymphoma.
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PMID:Primary macroglobulinemia with t(11;18)(q21;q21). 1070 80

Besides the problems inherent in endoscopically obtained tissue and the low incidence of intestinal lymphomas, the major difficulties reside in the distinction to reactive processes and in the differential diagnosis among several lymphoma entities. Knowledge of the microanatomical and biological properties of the intestinal MALT, supplemented by sufficient clinical information, are important prerequisites for the diagnostic work-up which has to include immunohistochemical studies. Whereas the diagnosis of aggressive B-cell lymphomas (diffuse large B-cell lymphoma, Burkitt's lymphoma) is usually straightforward, lymphomatous polyposis (LP, the intestinal equivalent of mantle cell lymphoma) and low-grade B-cell lymphoma of MALT-type may be difficult to diagnose and to separate from reactive lymphoproliferations. The characteristic immunohistochemical profile of LP (cyclin D1 + CD5 + CD43 + CD23 - CD10 - IgM kappa or lambda, is very helpful in this regard and similarly useful to exclude intestinal involvement by B-CLL or follicular center lymphoma. In addition, the endoscopic appearance characterized by seeds of small polyps along the colorectum favors LP although MALT-type lymphoma may occasionally produce polypoid lesions. Focal lymphoid hyperplasia occurs in the terminal ileum and may present with a mass in the right iliac fossa. The diagnosis of intestinal T-cell lymphoma (ITL) represents the most challenging task for both clinicians and pathologists. This disease is often associated with and may closely mimick celiac disease of adult onset type, or can be misdiagnosed as inflammatory bowel disease. The presence of an abnormal activated T-cell phenotype, i.e. different from that of normal intraepithelial lymphocytes, strongly suggests ITL and is of particular importance in cases that lack overt cytological atypia.
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PMID:[Problems in biopsy differential diagnosis in lymphomas of the small and large intestines]. 1071 99

The phenotypic characters of the lymphoma cell are important in the diagnosis of this disease. We recently tested whether the flow cytometry with fresh biopsy sample might be useful in the diagnosis of the lymphoma. In our date, 1. a rise and fall of the surface immunoglobulin kappa/lambda ratio indicated the monoclonal proliferation of the B-cell, 2. the increased proportion of the CD5/CD23 double positive cells indicated B-cell chronic lymphocytic leukemia or small lymphocytic lymphoma, 3. the decreased proportion of the CD2 positive cells and the increased proportion of the CD19 positive cells indicated B-cell lymphoma. These findings suggest that the flow cytometry is of adjunctive importance in making a diagnosis of the lymphoma.
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PMID:[Phenotypic classification of malignant lymphoma]. 1074 Nov 29

Primary cutaneous B-cell lymphomas (PCBLs) may have particular clinicopathologic characteristics distinct from their lymph node-based counterparts. It has been suggested that PCBLs should have a separate classification system. The aim of this study was to determine whether the Revised European-American Lymphoid Neoplasms (REAL) classification is applicable to PCBL. Thirty-nine cases of PCBL from 36 patients, consisting of 20 men and 16 women (median age 66 yrs), were included in this study. Paraffin-section immunohistochemistry for CD3, CD5, CD10, CD20, CD43, Bcl-2, Bcl-6, and cyclin D1 was performed in all cases. Immunostaining for immunoglobulin light chains was also performed on cases histologically diagnosed as extranodal marginal zone lymphoma (MZL) and primary cutaneous B-cell lymphoma unclassifiable (PCBLu). Polymerase chain reaction (PCR) analysis of t(14;18) was performed in all cases. Immunoglobulin heavy chain gene rearrangement (VDJ) was tested by PCR on all follicle center lymphoma (FCL), MZL, and PCBLu cases. The 39 cases consisted of 15 (39%) FCLs, 13 (33%) diffuse large B-cell lymphomas (DLCL), 9 (23%) extranodal MZL, and 2 cases of PCBLu. Anatomically, 59% of PCBLs occurred in the head and neck, of which approximately 57% were FCL. Five of six cases presenting on the lower extremity were DLCL. Follow-up data was available from all 39 patients with a mean of 50.8 months. All but two patients are alive with or without disease at last contact. One patient with DLCL died of lung metastases and the other DLCL patient died of sepsis as a complication of therapy. In all 15 cases of FCL, CD10 and/or Bcl-6 expression supported the follicle center origin of the neoplastic cells. In contrast to previous reports, we found that 53% (8 of 15) of primary cutaneous FCL had either Bcl-2 protein expression or t(14;18). Our data indicate that many cases of primary cutaneous FCL have Bcl-2 alterations similar to their nodal counterpart. We found that 95% (37 of 39) of PCBLs could be classified according to the REAL classification, supporting its applicability in cutaneous lymphomas.
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PMID:Clinicopathologic reassessment of primary cutaneous B-cell lymphomas with immunophenotypic and molecular genetic characterization. 1125 30

The clinical, histological, phenotypic and genotypic features of 21 primary cutaneous B-cell lymphomas (CBCLs) have been investigated. The patients were 13 men and eight women aged 34-91 years (median 67) at diagnosis. Eighteen patients had localized disease, and three had multiple skin lesions at diagnosis. Twelve patients developed cutaneous or extracutaneous recurrences, and five died from malignant lymphoma 7-84 months (median 36) after diagnosis. Histological examination showed features of marginal zone/mucosa-associated lymphoid tissue (MALT)-type lymphoma in 12 cases. Three of these had transformed to diffuse large B-cell lymphoma (DLBCL) in relapse biopsies. The remaining cases were seven primary DLBCLs and two cases tentatively classified as follicle centre cell (FCC) lymphoma. The neoplastic B cells showed similar phenotypes and genotypes in most cases (CD20+, CD79+, CD5-, CD10-, cyclin D1-, bcl-2+, bcl-x-, bax-, t(14;18)-negative). p53 protein was expressed in five cases, and four harboured mis-sense or loss-of-function mutations in the p53 gene. Deletion or promoter region hypermethylation of the p16INK4a gene was detected in two patients with DLBCL. The level of retinoblastoma protein expression and the proliferative fraction were significantly higher in DLBCL (> 50%) than in MALT- or FCC-type lymphomas (< 10%). Features associated with an unfavourable prognosis were the presence of multiple skin lesions at diagnosis, transformation from MALT-type lymphoma to DLBCL, and possibly p16INK4a aberrations. It is concluded that most CBCLs are dissimilar from FCC lymphomas and seem to be more closely related to marginal zone/MALT-type lymphomas. It is also suggested that there are fundamental differences between DLBCL and other histological categories of CBCL, indicating that cutaneous DLBCL is a separate entity with an increased growth potential and genetic features similar to DLBCL originating in other anatomical sites.
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PMID:Primary cutaneous B-cell lymphoma: a clinical, histological, phenotypic and genotypic study of 21 cases. 1080 48

B-cell lymphoma-associated hemophagocytic syndrome (B-LAHS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries, especially Japan. Here, we reviewed 25 previously reported Japanese cases of B-LAHS and summarized its clinicopathologic features and therapeutic outcome. The median age of onset was 63 years old with initial presentation of fever, hepatomegaly, and splenomegaly without associated lymphadenopathy. Laboratory findings showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Histopathologically, hemophagocytosis was often seen in the bone marrow and spleen. Various percentages of lymphoma cells were seen in the bone marrow, positive for CD19, CD20 and surface immunoglobulin. and some were also positive for CD5. Cytogenetic analysis showed a complex structural abnormality including chromosome 14q32, 19q13 and deletion of the terminal part of 8p21. Some patients had histological features of intravascular lymphomatosis (IVL). The prognosis was poor with a median survival period of 9 months. We treated five patients using autologous peripheral blood stem cell transplantation (PBSCT), and four are still in complete remission nine to 24 months after PBSCT, suggesting that high-dose chemotherapy followed by PBSCT might improve the survival rate.
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PMID:B-cell lymphoma-associated hemophagocytic syndrome. 1081 54

Primary cutaneous B-cell lymphomas (CBCLs) should be clearly separated from non-Hodgkin's B-cell lymphomas with secondary cutaneous involvement and from cutaneous B-cell pseudolymphomas. The majority of CBCLs are characterized by a homogeneous clinical presentation and behavior, with good response to local radiotherapy, low tendency to extracutaneous spread, and excellent prognosis. According to the European Organization for Research on the Treatment of Cancer classification of primary cutaneous lymphomas, CBCLs with an indolent behavior are divided into 2 subgroups: follicular center cell lymphoma and immunocytoma/marginal zone lymphoma, due to putative histologic similarities with their purported nodal counterparts. In addition, a third subgroup with intermediate prognosis (large B-cell lymphoma of the leg) is identified. Conversely, the identification of distinct subgroups is disputable from a strictly histologic, immunophenotypic, and genotypic point of view, and has neither correlation with the clinical course nor the prognosis of the disease. Moreover, the majority of CBCLs show a uniform immunophenotype (CD5-, CD10-) and genotype (lack of bcl-1/bcl-2 and c-myc gene rearrangement) of neoplastic cells. Therefore, we favor the use of the term Skin-Associated Lymphoid Tissue (SALT)-related B-cell lymphomas, due to the close similarities between CBCLs and mucosa-associated lymphoid tissue (MALT) lymphomas, and the evidence for an acquired B-cell arm of SALT.
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PMID:The skin-associated lymphoid tissue-related B-cell lymphomas. 1089 14

STAT3 is constitutively phosphorylated on tyrosine(705) in self-renewing, CD5(+) murine B-1 lymphocytes. Nuclear extracts from untreated primary B-1 or CD5(+) BCL(1) B lymphoma cells were found to contain immunoreactive STAT3 protein that binds to a sis-inducible element present in the promoter of the p21(waf1/cip1) tumor suppressor gene and is constitutively phosphorylated on serine(727). To determine the functional significance of constitutive STAT3 activation in B lymphoma cells, a specific STAT3 antisense oligonucleotide was developed and used to examine basal BCL(1) cell growth and IgM production. Abrogating STAT3 expression in BCL(1) cells inhibited their proliferative capacity and induced a corresponding decrease in secretion of IgM. Cell cycle analysis showed a block in progression through G1 in BCL(1) cells treated with the STAT3 antisense oligonucleotide. These results indicate that STAT3 controls cell growth and immunoglobulin secretion by enhancing progression through the G1 phase of the cell cycle in BCL(1) B cell lymphoma.
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PMID:STAT3 regulates the growth and immunoglobulin production of BCL(1) B cell lymphoma through control of cell cycle progression. 1089 72

The morphologic spectrum of large B-cell lymphoma is broad. Several unusual variants have been described such as lymphoma with myxoid stroma, sclerosing B-cell lymphoma, signet ring-cell lymphoma, and multilobated B-cell lymphoma among others. We report on five cases of cutaneous large B-cell lymphoma in which the neoplastic cells were spindle-shaped. In two cases, the clinical features fulfilled those of a primary cutaneous lymphoma; in the three other cases, the lymphoma most likely arose primarily in the skin, but incomplete clinical workups precluded definite categorization. The patients ranged in age from 30 to 89 years and presented with solitary lesions on the trunk or head. Histopathologic examination revealed nodular or dense diffuse infiltrates involving the entire dermis as well as the subcutaneous fat in some cases. Thickened collagen bundles between the spindled cells were present in one case. Cytomorphologic analysis showed the presence of round or oval medium-sized and large-sized lymphocytes with features of centrocytes and centroblasts in some foci, with others dominated by cells with spindle-shaped, elongated, twisted nuclei with dispersed chromatin and scant cytoplasm. Immunohistologic analysis revealed that both round or oval and spindled cells were positive for CD20 in all cases; in all cases tested, these cells were also positive for MIB-1 and were negative for CD3, CD5, CD43, CD45RO, CD21, CD30, CD68, S-100, HMB-45, actin, smooth-muscle actin, and cytokeratin. Bcl-2 was expressed in one of three cases tested. Analysis of the rearrangement of the J(H) gene by polymerase chain reaction performed in one case showed a monoclonal pattern. Spindle-cell large B-cell lymphoma represents a distinctive rare subtype of the cutaneous large B-cell lymphoma and can arise primarily in the skin in some cases. Recognition of this variant is necessary to avoid misdiagnosis of other cutaneous malignant spindle-cell tumors.
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PMID:Cutaneous spindle-cell B-cell lymphoma: a morphologic variant of cutaneous large B-cell lymphoma. 1245 11

Seven patients with peripheral B-cell lymphoma associated with hemophagocytic syndrome are reported. In all cases, the histologic subtype was diffuse large B-cell lymphoma. Hemophagocytic features were noted in the bone marrow with lymphomatous infiltration. Hemophagocytic syndrome occurred with presentation of the lymphoma and was characterized by high fever, cytopenias, and elevated levels of lactate dehydrogenase, ferritin, C-reactive protein, and cytokines [interferon gamma, macrophage colony-stimulating factor, soluble interleukin (sIL)-2R, and IL-6] without evidence of infection. The phenotypes of lymphomas were suspected CD19+, CD20+, S-Ig+, CD10-, and coexpression of CD5 in some cases. Flow cytometric analysis showed a low CD4/CD8 ratio in peripheral blood and bone marrow. We suggest that the pathogenesis of hemophagocytic syndrome is hypercytokinemia induced by a proliferation of reactive CD8+ T cells. Previous reports of B-cell lymphoma with hemophagocytic syndrome demonstrated similar clinical manifestations and poor prognoses. The invasion patterns of these diffuse large B-cell lymphomas with hemophagocytosis may be classified into three groups: microscopic lymph-node involvement type, gross lymph-node involvement type, and splenic lymphoma type. Although hemophagocytic syndromes have been reported to be associated with T-cell lymphomas, our results indicate an association with diffuse large B-cell lymphoma.
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PMID:B-cell lymphoma-associated hemophagocytic syndrome: clinicopathological characteristics. 1096 86


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