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)

Clinicopathological and immunophenotypical characteristics in 24 patients with diffuse large B-cell lymphoma (DLBCL) under 30 years of age in Osaka, Japan were examined, and the results compared to those of DLBCL patients aged over 40 years in Osaka and of young DLBCL patients in Western countries. The level of LDH and IPI score at initial diagnosis were significantly lower in young than older patients. The sex ratio (M:F) and age range (median) in the young and older groups were 1.18 and 11-30 (24.8) years and 1.59 and 42-87 (62.4) years, respectively. Extranodal presentation was higher in the young group (83.3% versus 60.0%, P < 0.05). Based on immunophenotyping with anti-CD10, bcl-6, and MUM1 antibodies, the cases were categorized as germinal center B-cell (GCB) (CD10+ or CD10-, bcl-6+, MUM1+) or non-GCB phenotype. The frequency of GCB type was significantly lower in the young group than older group (25% vs. 54%, P < 0.05), and much lower than that reported for young patients in Western countries. In situ hybridization revealed one of the young patients to be positive for Epstein-Barr virus (EBV). In the older group, none of 31 cases showed EBV positivity. Three year event-free and overall survival rates of young patients were better than those of the older patients, although not significantly different. DLBCL in the young in Japan is characterized by a much lower frequency of the GCB phenotype compared to that in Western countries.
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PMID:Diffuse large B-cell lymphoma in the young in Japan: a study by the Osaka Lymphoma Study Group. 1757 93

Polo-like kinase 1 (PLK1) belongs to a conservative family of serine/threonine kinase and plays an important role in the process of cell cycle. Survivin is a member of inhibitor of apoptosis protein (IAP) family. We investigated the expression of PLK1 and survivin with immunohistochemical techniques in diffuse large B-cell lymphoma (DLBCL) and assessed their significance as a potent new tumor marker. The expression rate of PLK1 and survivin were 66.7% (26/39) and 82.1% (32/39), respectively. PLK1 expression correlated with systemic symptom, LDH level, IPI scores and therapeutic effect in DLBCL, while survivin did not. PLK1 expression correlated with shortened event-free survival (EFS) using the Log-rank test in DLBCL, but survivin did not. Cox regression analysis identified the independent prognostic significance for PLK1. The results suggest that there is a significant relationship between over expression of PLK1, the clinical features and survival time. Compared with survivin, PLK1 seems to be a better independent prognostic factor for DLBCL.
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PMID:Expression of PLK1 and survivin in diffuse large B-cell lymphoma. 1833 44

A 69-year-old male was diagnosed in February 2004 with stage IV extranodal marginal zone B cell lymphoma involving the mediastinal nodes, lung parenchyma and bone marrow with high LDH. Shortness of breath developed following the 5th course of Rituximab-CHOP chemotherapy (cyclophosphamide, Vincristine, Doxorubicin, Prednisolone). Bronchoscopy guided transbronchial lung biopsy revealed interstitial thickening and type II pneumocyte activation, compatible with interstitial pneumonitis. After treatment with prednisolone a complete resolution of the dyspnea was observed. The patient was well on routine follow-up at the outpatient clinic, with no progression of lymphoma or interstitial pneumonitis.
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PMID:Rituximab-CHOP induced interstitial pneumonitis in patients with disseminated extranodal marginal zone B cell lymphoma. 1830 83

Early diagnosis of intravascular large B-cell lymphoma (IVLBCL) is difficult, but is critical for longer survival for the patients. We report a case of IVLBCL that was diagnosed with the help of FDG-PET. A 76-year-old woman was referred to us for the evaluation of her elevated serum LDH. She presented with general malaise and high fever. There were no skin lesions or neurological involvement. FDG-PET imaging showed increased uptake of FDG in the vertebra, bilateral femurs, sternum, and iliac bones. A diagnosis of IVLBCL was made by bone marrow biopsy. She was successfully treated with rituximab and modified CHOP therapy.
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PMID:Successful treatment of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy and FDG-PET scan. 1848 May 85

Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large B-cell lymphoma. It is characterized by proliferation of neoplastic Lymphoid cells almost exclusively within the lumina of small blood vessels. It can affect virtually every organ system. Due to its rarity and its diverse and heterogeneous clinical presentation, diagnosis is difficult and often made post-mortem. When diagnosed early, it is, however, potentially treatable. We present a young woman with longstanding constitutional symptoms, positive antinuclear antibody, elevated LDH levels and rapidly progressive encephalopathy. FDG-PET scan showed intense uptake in the renal cortex, which prompted us to perform a kidney biopsy which was compatible with IVL. The value of PET in establishing the diagnosis of this rare disease will be discussed.
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PMID:Usefulness of FDG-PET to diagnose intravascular lymphoma with encephalopathy and renal involvement. 1871 49

Primary mediastinal large B-cell lymphoma (PMLBCL) is a unique type of B-cell lymphoma probably arising from a putative thymic medulla B-cell. It constitutes 6-10% of all diffuse large B-cell lymphomas (DLBCL), occurring more often in young females. PMLBCL is characterized by a diffuse proliferation of medium to large B-cells associated with sclerosis and a degree of compartmentalisation. Its main molecular characteristics include: gains in 9p segments, p53 mutations, BCL-2 and MAL gene over-expression, somatic mutations of IgVH genes, BCL-6, PIM-1, PAX-5, RhoH/TTF, and c-MYC, and constitutional NF-kappaB activation. The gene expression signature of PMLBCL seems to be much closer to classic Hodgkin lymphoma than to DLBCL. PMLBCL is characterized by a locally invasive anterior mediastinal mass, often producing cough, chest pain, dyspnea, and superior vena cava syndrome. Most PMLBCL patients have stage I-II, bulky disease, with pleural or pericardial effusions in a third of cases. Systemic symptoms, mainly fever or weight loss, are present in <20% of cases; increased LDH levels are observed in 70-80% of cases. Treatment with CHOP regimen followed by radiation therapy was associated with a 5-year survival of 65%. Apparently better results have been reported with third-generation weekly alternating regimens followed by radiation therapy. Any recurrence is almost always seen in the first 2 years of follow-up, and distant relapses tend to involve extranodal organs. Features associated with poor prognosis are poor performance status, pericardial effusion, bulky disease, high serum LDH at diagnosis, and a compromised dose-intensity of anthracycline and cyclophosphamide.
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PMID:Primary mediastinal large B-cell lymphoma. 1877 28

Clinical studies showed that advanced stage, high LDH, poor response to reduction therapy and combined bone marrow and central nervous system disease are significantly associated with a decreased event-free survival (EFS) in pediatric mature B-cell non-Hodgkin's lymphoma (B-NHL) treated on FAB/LMB96. Although rearranged MYC/8q24 (R8q24) is characteristic of Burkitt lymphoma (BL), little information is available on other cytogenetic abnormalities and their prognostic importance. We performed an international review of 238 abnormal karyotypes in childhood mature B-NHL treated on FAB/LMB96: 76% BL, 8% Burkitt-like lymphoma, 13% diffuse large B-cell lymphoma (DLBCL). The main BL R8q24-associated chromosomal aberrations were +1q (29%), +7q and del(13q) (14% each). The DLBCL appeared heterogeneous and more complex. Incidence of R8q24 (34%) was higher than reported in adult DLBCL. The prognostic value of cytogenetic abnormalities on EFS was studied by Cox model controlling for the known risk factors: R8q24, +7q and del(13q) were independently associated with a significant inferior EFS (hazard ratio: 6.1 (P=0.030), 2.5 (P=0.015) and 4.0 (P=0.0003), respectively). The adverse prognosis of R8q24 was observed only in DLBCL, whereas del(13q) and +7q had a similar effect in DLBCL and BL. These results emphasize the significant biological heterogeneity and the development of cytogenetic risk-adapted therapy in childhood mature B-NHL.
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PMID:Specific cytogenetic abnormalities are associated with a significantly inferior outcome in children and adolescents with mature B-cell non-Hodgkin's lymphoma: results of the FAB/LMB 96 international study. 1902 May 48

The literature on adult and pediatric primary mediastinal B-cell lymphoma (PMBCL) was reviewed and compared. Biologically, adult PMBCL has more similarities to Hodgkin Lymphoma (HL) than diffuse large B-cell lymphoma (DLBCL). Pediatric studies suggest that the biology is similar to that in adults. Median age of children is 14.3 years and the overall survival (OS) is reported as 78.6% and event-free survival (EFS) as 67.4%. Adverse prognostic factors included LDH >500 and mass size over 10 cm, with a trend towards better survival in younger patients. Studies in adults show better survival with intensified chemotherapy and the addition of rituximab. Data on the use of radiation therapy show improved CR rates and survival with addition of involved field radiation therapy (IFRT). Positron emission tomography (PET) with computerized tomography (CT) imaging response-assessment after two courses and at therapy-end may allow for the rational use of IFRT in pediatric/adolescent patients who are more susceptible to development of adverse late effects.
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PMID:Primary mediastinal B-cell lymphoma in the pediatric patient: Can a rational approach to therapy be based on adult studies? 1905 8

Richter syndrome (RS) represents the clinico-pathologic transformation of chronic lymphocytic leukaemia (CLL) to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). The clinical definition of RS is heterogeneous, and encompasses at least two biologically different conditions: (i) CLL transformation to a clonally related DLBCL, that accounts for the majority of cases; (ii) development of a DLBCL unrelated to the CLL clone. In clonally related RS, the pathogenetic link between the CLL and the DLBCL phases is substantiated by the acquisition of novel molecular lesions at the time of clinico-pathologic transformation. RS is not a rare event in the natural history of CLL, since the cumulative incidence of RS at 10 years exceeds 10%. Prompt recognition of RS is known to be clinically useful, and may be favoured by close monitoring of CLL patients harbouring clinical and/or biological risk factors of RS development. Conventional risk factors that are independent predictors of RS development at the time of CLL diagnosis include: (i) expression of CD38; (ii) absence of del13q14 and (iii) lymph node size > or =3 cm. Other risk factors of RS development include CD38 genotype and usage of specific immunoglobulin variable genes. The molecular pathogenesis of RS has been elucidated to a certain extent. Acquisition of TP53 mutations and/or 17p13 deletion is a frequent molecular event in RS, as it is in other types of transformation from indolent to aggressive B-cell malignancies. Additional molecular alterations are being revealed by genome wide studies. Once that transformation has occurred, RS prognosis may be predicted by the RS score, based on performance status, LDH, platelet count, tumour size and number of prior therapies. Depending on patient's age and RS score, the therapeutic options for RS may range from conventional immunochemotherapy to allogeneic bone marrow transplantation.
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PMID:Richter syndrome: molecular insights and clinical perspectives. 1920 12

Rituximab has been associated with the development of late-onset neutropenia (LON). As only heterogeneous studies have been conducted, its incidence and clinical course remain unclear. We aim to: (1) study the incidence and clinical relevance of WHO grade 3/4 LON in a uniform group of patients with diffuse large B-cell lymphoma (DLBCL) in complete remission following curative rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (RCHOP) chemotherapy; (2) ascertain predictive factors for LON. The 121 eligible patients identified from our prospectively maintained database were followed up for occurrence of WHO grade 3/4 LON. The clinical course of LON was documented, and its relationship with patient- and tumor-related factors was analyzed. With a median follow-up of 883 days (range, 265-1762), 13.2% had developed LON of grade 3/4. The median time to neutrophil nadir was 129 days (range, 39-277). The median time to recovery was 69 days (range, 3-349) and occurred in all except two patients. Only one episode of nonlife threatening bacterial culture-positive urinary tract infection and pulmonary tuberculosis, both occurring in the same patient was documented. Results of Fischer's exact test revealed that age, stage, LDH level, ECOG, marrow involvement, and hematologic parameters did not predict for LON development. WHO grade 3/4 LON is not infrequent in patients with DLBCL receiving RCHOP. Even so, it is reassuring that LON is self-limiting and unassociated with life-threatening infection. A watchful waiting approach is appropriate in majority of patients who develop LON following RCHOP. Am. J. Hematol., 2009. (c) 2009 Wiley-Liss, Inc.
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PMID:Late-onset neutropenia following RCHOP chemotherapy in diffuse large B-cell lymphoma. 1941 27


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