Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Posttransplantation lymphoproliferative disorders (PTLDs) represent a serious complication of solid organ transplantation. This study assessed the molecular histogenesis of 52 B-cell monoclonal PTLDs, including 12 polymorphic PTLDs (P-PTLDs), 36 diffuse large B-cell lymphomas (DLBCLs), and 4 Burkitt/Burkitt-like lymphomas (BL/BLLs). Somatic hypermutation (SHM) of immunoglobulin variable (IgV) genes documented that most monoclonal B-cell PTLDs (75% P-PTLDs, 91.3% DLBCLs, 100% BL/BLLs) derive from germinal center (GC)-experienced B cells. B-cell lymphoma 6 (BCL6) mutations occurred in 25% P-PTLDs, 60.6% DLBCLs, and 75.0% BL/BLLs. A first histogenetic category of PTLDs (31.2% DLBCLs) express the BCL6+/multiple myeloma oncogene-1 protein (MUM1-/+)/CD138- profile and mimic B cells experiencing the GC reaction, as also suggested by ongoing SHM in a fraction of these cases. A second subset of PTLDs (66.7% P-PTLDs and 31.2% DLBCLs) display the BCL6-/MUM1+/CD138- phenotype and mimic B cells that have concluded the GC reaction. A third histogenetic category of PTLDs (25.0% P-PTLDs and 31.2% DLBCLs) shows the BCL6-/MUM1+/CD138+ profile, consistent with preterminally differentiated post-GC B cells. Crippling mutations of IgV heavy chain (IgVH) and/or IgV light chain (IgVL) genes, leading to sterile rearrangements and normally preventing cell survival, occur in 4 DLBCLs and 1 BL/BLL that may have been rescued from apoptosis through expression of Epstein-Barr virus (EBV)-encoded latent membrane protein 1 (LMP1). Overall, the histogenetic diversity of monoclonal B-cell PTLDs may help define biologically homogeneous categories of the disease.
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PMID:Molecular histogenesis of posttransplantation lymphoproliferative disorders. 1290 42

The aim of the present study was to estimate optimum chemotherapeutic regimens for high-grade mature B-cell lymphoma cases with Burkitt-like morphology (Burkitt's lymphoma [BL]/Burkitt-like lymphoma [BLL]) patients. We analyzed 72 BL/BLL, including 36 with the c-myc translocation (molecular BL [mBL]), 20 without it (mBL-like), and 16 in whom we were uncertain regarding the existence of the c-myc translocation, and compared them with 182 diffuse large B-cell lymphoma (DLBCL) cases. On clinical and immunophenotypic analysis, the typical BL immunophenotype (CD10 positive, bcl-2 negative, and Ki-67 index >or=95%) was noted in 23 (66%) and 11 (55%) of the 35 mBL and 20 mBL-like patients, respectively. The presence of the c-myc translocation and typical immunophenotype in BL did not affect the overall survival of BL/BLL. There were no significant differences between the overall survival of DLBCL (45%) and BL/BLL (50%, P = 0.85). However, the overall survival of BL/BLL patients who received cyclophosphamide, doxorubicin, vincristine, and prednisolone-related therapy (22%) was significantly lower than that of DLBCL patients (P = 0.01). In contrast, the overall survival of BL/BLL patients who received aggressive short-term chemotherapy (75%) was better than that of the patients who received cyclophosphamide, doxorubicin, vincristine, and prednisolone therapy (P < 0.01). The finding was confirmed by multivariate analysis (hazard ratio 4.4; confidence interval 2.0-9.7; P = 0.0003). We concluded that aggressive short-term chemotherapy improves survival in BL/BLL, regardless of its genetic and immunophenotypic features.
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PMID:High-grade mature B-cell lymphoma with Burkitt-like morphology: results of a clinicopathological study of 72 Japanese patients. 1827 22

We report a rare case of primary cutaneous diffuse large B-cell lymphoma (DLBCL) with Burkitt-like morphology. A 54-year-old man presented with multiple subcutaneous tumors. Pathological examination showed morphological features resembling Burkitt or Burkitt-like lymphoma (BL/BLL) with high MIB-1 positivity. Cytogenetic studies revealed no 8q24/c-myc translocation. After the diagnosis of Burkitt-like DLBCL, the patient was treated with CODOX-M chemotherapy (cyclophosphamide, doxorubicin, vincristine, cytarabine and methotrexate), which led to durable remission. The present case suggests that short-term, high-intensity chemotherapy used for BL/BLL may be appropriate for primary cutaneous Burkitt-like DLBCL, as well as systemic lymphoma with Burkitt-like morphology.
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PMID:Intensive chemotherapy for a patient with primary cutaneous diffuse large B-cell lymphoma with Burkitt-like morphology. 1929 50

Burkitt lymphoma (BL) is a mature B-cell non-Hodgkin lymphoma with an aggressive clinical course. Since the advent of short, intensive, multiagent chemoimmunotherapy regimens, it has carried a favorable prognosis. BL has been rather well characterized, whereas the other lymphomas morphologically resembling it are more heterogeneous. The cases classified as atypical BL/Burkitt-like lymphoma by the 2001 World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissue were thought to represent a continuum between BL and diffuse large B-cell lymphoma (DLBCL). The optimal therapeutic strategy for this provisional entity was not definitively established. However, recent incorporation of molecular genetic data into the 2008 WHO Classification has allowed further refinements with significant therapeutic implications, including the designation of a new provisional entity, "B-cell lymphoma, unclassifiable, with features intermediate between BL and DLBCL." This review presents a comprehensive overview of the previously designated provisional entity of atypical BL/BLL in conjunction with a detailed comparison with BL and DLBCL.
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PMID:Burkitt lymphoma and atypical Burkitt or Burkitt-like lymphoma: should these be treated as different diseases? 2119 75