Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Trisomy 3 has been reported to be associated with marginal zone B-cell lymphoma. However, its occurrence and significance in other B-cell chronic lymphoproliferative disorders has not been fully defined. We report five cases of B-cell chronic lymphoproliferative disorders showing gain of chromosome 3 or 3q. The patients were elderly males who presented with splenomegaly with or without hepatomegaly and lymphadenopathy. The diagnoses included chronic lymphocytic leukemia (3 cases), prolymphocytic leukemia (1 case), and Waldenstrom macroglobulinemia (1 case). Distinctive feature in this group of patients was the plasmacytoid appearance of the leukemic lymphocytes, with an associated IgM hypergammaglobulinemia in three patients. The relationship between the gain of chromosome 3 and plasmacytoid differentiation in B-cell chronic lymphoproliferative disorders is discussed.
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PMID:Gain of chromosome 3/3q in B-cell chronic lymphoproliferative disorder is associated with plasmacytoid differentiation with or without IgM overproduction. 1216 58

Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade B-cell lymphoma defined in the World Health Organization classification as the leukaemic form of splenic marginal zone lymphoma. Presenting features and response to therapy have been described, but information on prognostic factors is scanty. Clinical, laboratory and follow-up data were collected on 129 patients with SLVL to determine features predicting disease behaviour and survival. Diagnosis was made on clinical, morphological and immunophenotypic features and, where available, bone marrow and spleen histology. Median age was 69 years (range 39-90 years) and male:female ratio, 0.9. The majority had splenomegaly, but lymphadenopathy and hepatomegaly were rare. Median Hb was 11.8 g/dl, white blood cell count was 16 x 10(9)/l and platelet count was 145 x 10(9)/l; 27% of patients had monoclonal protein in serum and/or urine. While 27% of patients remained untreated, 10% transformed to high-grade lymphoma. Median follow-up was 61 months and median survival was 13 years, with 72% of patients alive at 5 years. Cox regression analysis showed that increasing age, anaemia, thrombocytopenia and lymphocytosis > 16 x 10(9)/l were independent adverse predictors of overall survival. However, only anaemia and lymphocytosis > 16 x 10(9)/l remained highly significant independent prognostic factors when only deaths due to lymphoma were analysed. Splenectomized patients fared better than those receiving chemotherapy only (P = 0.001 for SLVL deaths). We conclude that SLVL is mainly a disease of the elderly with a relatively benign course but, when treatment is required, splenectomy is beneficial.
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PMID:Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients. 1453 26

Clinicopathologic features of 21 patients with T-cell-rich B-cell lymphoma (TCRBCL) were reviewed and compared to 43 patients with diffuse large B-cell lymphoma (DLBCL) to determine if there were distinguishing clinical characteristics and differences in response or survival to CHOP therapy. For the diagnosis of TCRBCL, the current WHO criteria was used. In all of our cases, the majority of cells are non-neoplastic T cells and <10% large neoplastic B cells are present. The initial pathologic diagnosis was nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) in two cases. Patients with TCRBCL were significantly younger (median: 46 years) and had a significantly higher incidence of B symptoms (62%), hepatomegaly (33%) and marrow infiltration (33%) at presentation when compared to DLBCL (P<0.03). The CR rate after treatment was 48% for TCRBCL patients versus 79% for the DLBCL (P<0.003). Although the CR rates in between the two groups are significant, the difference in 3 years survival rates in each CR groups was insignificant (80% versus 77%). The overall survival time in the two groups was 17 months. Event-free survival time in TCRBCL was 12 months, compared with 17 months in the DLBCL (P>0.05). The frequency of patients with TCRBCL achieving CR was 52.6% whereas that of patients with DLBCL was 79% (P<0.003). The TCRBCL 3 years event-free survival 48% and overall survival 64% were 63 and 72% for DLBCL, respectively.
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PMID:T-cell-rich B-cell lymphoma: a clinicopathologic study of 21 cases and comparison with 43 cases of diffuse large B-cell lymphoma. 1468 17

Hepatic lymphoma metastasis is rare, and should always be considered in the differential diagnosis of hepatic malignancy. A 52-year-old man presented with a four-day history of fever, fatigue, yellowish skin and nausea. His past medical history was unremarkable. There was no history of alcohol intake or medications. His physical examination revealed generalized jaundice and hepatomegaly. His blood tests showed liver failure and coagulopathy. Abdominal ultrasonography illustrated hepatomegaly. A further work-up included bone marrow and liver biopsy. The pathology report was B-cell lymphoma. He was treated with chemotherapy, and his laboratory findings during follow-up showed steady improvement. In conclusion, lymphoma metastasis to liver can be a cause of liver dysfunction. A high index of suspicion is required for the diagnosis. We emphasize the importance of obtaining tissue sample in all patients with suspicious lesion in any organ to avoid missing the rare but curable pathologies.
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PMID:Hepatic lymphoma metastasis presenting with severe acute liver failure: a rare case. 1533 18

We review the literature on primary hepatic lymphoma (PHL). PHL is a rare malignancy, and constitutes about 0.016% of all cases of non-Hodgkin's lymphoma. It has been reported to occur with increased frequency in patients with chronic hepatitis C infection. Most patients with PHL present with abdominal pain, constitutional symptoms and have hepatomegaly on examination. Imaging studies reveal solitary, or less often, multiple masses in the liver. The predominant histology is B-cell lymphoma, most commonly diffuse large cell type. Most patients are treated with chemotherapy, with some physicians employing a multimodality approach incorporating surgery and radiotherapy with chemotherapy. The prognosis is variable, with good response to early aggressive combination chemotherapy.
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PMID:Primary non-Hodgkin's lymphoma of the liver. 1571 46

Disorders of the cell cycle regulatory machinery play a key role in the pathogenesis of cancer. Over-expression of cyclin D1 protein has been reported in several solid tumors and certain lymphoid malignancies, but little is known about the effect of its expression on clinical behavior and outcome in B-cell Non-Hodgkin lymphoma (NHL). In this study, we investigated the expression of cyclin Dl in group of patients with NHL and correlated the results with the clinical and laboratory data. The degree of expression of cyclin Dl protein was evaluated by flow cytometry in a group of NHL patients (n = 46) and in normal control group (n = 10). Cyclin Dl over expression was detected in 10 out of 46 (21.7%) patients; they were 5/5-mantle cell lymphoma (MCL) (100%) and 5/28 large B-cell lymphoma (17.8%). All other NHL subtypes showed normal cyclin D1 expression. The clinical signs (hepatomegaly, splenomegaly and B-symptoms, clinical staging) and laboratory data (hemoglobin, white cell count (WBCs), platelet count, and bone marrow infiltration) were not significantly different between NHL subgroup with cyclin Dl over expression and that with normal cyclin Dl expression. Serum lactic dehydrogenase (LDH) levels and lymphadenopathy were significantly higher in NHL group with cyclin D1 over expression as compared to those without. Also, cyclin D1 over expression is associated with poor outcome of NHL patients. Cyclin Dl over expression was evident among all cases of MCL and few cases of large B-cell lymphoma. Cyclin Dl over expression might be used as adjuvant tool for diagnosis of MCL; has role in NHL biology and is bad prognostic index in NHL.
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PMID:Cyclin Dl expression in B-cell non Hodgkin lymphoma. 1760 88

Immunocytoma is a non-Hodgkin's indolent evolution B cell lymphoma. It accounts for approximately 1-3% of non-Hodgkin's limphomas and usually onsets in adults aged over 50 years old. It manifests as lymphadenopathy, splenomegaly, hepatomegaly and lymphcytosis in 15 -30% of cases and is rarely seen with pulmonary involvement. Monocloncal peaks of serum immunoglobulin often occur. These are IgM and rarely IgA. We present as an example a male patient aged 52 years old, with recurrent respiratory infections. Clinical work -up identified an immunocytoma IgA stage IV. Diagnosing an indolent lymphoma, we prophylactic polyspecific human immunoglobulin to treat the respiratory infection. Evidence of lymphoma progression leads us to prescribe combined cyclo- phosphamide (C), vincristine (V), prednisone (P) e rituximab (R) (CVP-R), which has obtained a partial response over two years.
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PMID:[Immunocytoma IgA. Case report]. 1914 95

A 91-year-old woman presented with a rapidly proliferative cutaneous lesion on the left lower limb, which was identified as a primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, on biopsy. The patient also showed complications of hepatomegaly, endocrinopathy, edema, skin change, and polyneuropathy without monoclonal plasma cell proliferative disorder, and was therefore diagnosed with POEMS-like syndrome owing to the lack of monoclonal plasma cell proliferative disorder. Levels of serum vascular endothelial growth factor (VEGF) and interleukin-6 (IL-6) were high with the lymphoma cells immunostained positively for VEGF and IL-6. To the best of our knowledge, this is the first case report of PCLBCL, leg type, with POEMS-like syndrome. The findings in this case suggest that the symptoms of POEMS-like syndrome might be caused by the cytokines produced by the lymphoma cells. Furthermore, a wider range of diagnostic criteria associated with the result of abnormal secretion of cytokine may have to be considered for the diagnosis and evaluation of patients with possible POEMS syndrome, as against the present criteria specifying monoclonal plasma cell proliferative disorder as the essential criterion.
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PMID:Primary cutaneous diffuse large B-cell lymphoma, leg type, with features simulating POEMS syndrome. 1955 4

We present the case of a 65 years old male, admitted in the Hematology Department of the Universitary Emergency Hospital Bucharest, complaining of physical asthenia and weight loss; periodical medical examination has revealed splenomegaly and leucocytosis with lymphocytosis, persistent for the past 3 years. The clinical and paraclinical exam demonstrated splenomegaly (21 cm in diameter on computer tomography scan), hepatomegaly and generalized lymphadenopathies. The laboratory tests confirmed leucocytosis with lymphocytosis--a clonal population of B lymphocytes CD20+ CD19+ CD23+/- CD79b+(low), CD43+ FMC7+ CD5+ CD38+ ZAP70+ cyclin D1-. Lymph node and bone marrow biopsy together with flowcytometry established the diagnosis of Malignant non-Hodgkin Lymphoma--Atypical Splenic Marginal Zone B-cell lymphoma (aberrant expression of CD5) stage IVB, with leukemic picture, complicated with autoimmune hemolytic anemia with highly positive Coombs' tests. We performed therapeutic splenectomy, which was difficult because of the dimensions of the organ. The short term evolution was complicated by acute complete thrombosis of the splenic vein, but the long term evolution (1 year follow-up) was favorable--remission of anemia, significant improvement of performance status, decrease of leucocytosis and reduction of the tumoral mass.
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PMID:Splenectomy--a therapeutic option in splenic marginal zone cell lymphoma. 2006 70

A 13-year-old mixed-breed dog showing ascites, anorexia and anemia was found to have leukocytosis with marked eosinophilia, splenomegaly and hepatomegaly. The dog died 4 days after initial presentation and was diagnosed with splenic high-grade B-cell lymphoma at necropsy. Remarkable infiltrations of eosinophils were observed in spleen and liver tissues. The eosinophilia and infiltration of eosinophils into the lesions could have been associated with B-cell lymphoma because causes other than lymphoma were excluded. This is the first report of eosinophilia and eosinophilic infiltrations into neoplastic lesions in a dog with high-grade B-cell lymphoma.
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PMID:Eosinophilia and eosinophilic infiltration into splenic B-cell high-grade lymphoma in a dog. 2046 2


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