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)

In 1992, after a history of more than two decades a subgroup within the diffuse low-grade B cell lymphomas designated centrocytic lymphoma, lymphocytic lymphoma of intermediate differentiation or mantle zone lymphoma gained general acceptance, now referred to as mantle cell lymphoma. Similarities between these entities were emphasized by identification of rearrangement and overexpression of CCND1 (bcl1/PRAD1) gene in the majority of cases. Unlike in all other non-Hodgkin's lymphomas sex distribution demonstrates a striking preponderance of males over females with a ratio of 3:1. Initial parameters in all published series are advanced disease with generalized lymphadenopathy in 90%, bone marrow infiltration in 60-75%, splenomegaly in 55%, hepatomegaly in 35%, gastrointestinal involvement in about 25% and peripheral blood lymphocytosis in 20-30% of patients. In generalized disease, clinical course is characterized by continuous progression with a median survival probability of 3-4 years within most series. Overall response rates of 56-88% with complete remissions in the range of 9-58% are attainable but relapse occurs predominantly within 20 months. At present there is no evidence that any conventional regimen is curative. Prospective multicenter studies are mandatory to overcome this therapeutic dilemma. Patients suitable for some form of maintenance or consolidation therapy should initially be treated intensively by anthracycline-containing regimens. Whether maintenance with interferon or intermittent chemotherapy including new agents, like purine analogues or (un)conjugated monoclonal antibodies are able to influence overall survival is a matter of (ongoing) investigations. Further experimental approaches arise from antisense oligonucleotides or ribozymes blocking the overexpression of bcl-1 especially in this lymphoma entity. At present high-dose myeloablative consolidation radiochemotherapy followed by stem cell rescue in first remission seems to be the most attractive option in younger patients.
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PMID:Mantle cell lymphoma: diagnostic criteria, clinical aspects and therapeutic problems. 917 43

The authors present the characteristics of a group of 23 patients with mantle cell lymphoma. In the group only a slight predominance of men over women was found (1.1:1), the median age was 63 years. Twenty-one (91%) of the patients were diagnosed in stage IV (Ann Arbor). In all these patients the bone marrow was affected. In 19 of them immunoflowcytometric analysis revealed the typical clone of B lymphocytes (CD5 positive)/CD 23 negative). The majority of patients had at the time of diagnosis a large tumourous mass with massive splenomegaly (61%), hepatomegaly (57%) and bulky disease (52%). The node was excised in 17 patients, but in four patients (24%) during the first session the diagnosis was not assessed correctly. In the laboratory findings an inclination to anaemia, thrombocytopenia, lymphocytosis and in particular to high levels of serological indicators of activity of the disease dominated--lactate dehydrogenase, beta-2-microglobulin and serum thymidine kinase. All patients were treated by chemotherapy. Complete remission was achieved by the date of evaluation in one patient (4%), partial remission in seven patients (30%) but 48% patients did not respond to first line treatment. Nine patients of the group died, their median of survival was 14 months (0-24), the median of the follow up of the remaining patients was 133 months (2-31). Two female patients had large-dose treatment with subsequent administration of autologous stem cells. The first one is after 370 days of treatment in complete remission, the second one developed a relapse 100 days after the procedure. From the results and analysis of the literature ensues that mantle cell lymphoma is one of the aggressive malignant B-lymphoproliferations with a very adverse prognosis and it deserves therefore special diagnostic and intense therapeutic attention.
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PMID:[Mantle cell lymphoma as a diagnostic and therapeutic problem]. 1042 28

A 76-year-old man presented with leukostasis syndrome, including oculodynia, blurred vision, and visual field defects, due to mantle cell lymphoma, prolymphocytoid variant, with marked leukocytosis, 1227 x 10(9)/l. He had splenomegaly but no lymphadenopathy or hepatomegaly. The tumor cells were CD5+, CD19+, CD20+, FMC-7+, and kappa light chain restricted. Immunohistochemistry showed expression of p53 and of cyclin D1. Fluorescent in situ hybridization demonstrated t(11;14) with translocation between CYCLIN D1 and the immunoglobulin heavy-chain genes. The patient received leukapheresis and aggressive chemotherapy, but the leukocyte count remained above 100 x 10(9)/l. The patient's condition rapidly deteriorated with lymphomatous infiltration of his lungs and soft tissues, and he expired 6 months after diagnosis. While it is known that mantle cell lymphoma may have a leukemic phase, the degree of leukocytosis in this case exceeds that previously reported in the literature and resulted in a clinical syndrome of leukostasis.
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PMID:Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome. 1519 1

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

Leukaemic phase of non-Hodgkin lymphoma (NHL) is characterised by penetration of lymphoma cells from the originating tissues (lymph nodes, less commonly the spleen) into the peripheral blood and bone marrow. The diagnosis of leukaemic phase of Mantle zone lymphoma is established on the basis of histological findings of lymph node biopsy and, possibly, the spleen, peripheral blood smear, and characteristic membranous phenotype. A patient, aged 60, is reported with Mantle zone (intermediate lymphoma) in leukaemic phase. Physical examination revealed pallor of the skin, generalized lymphadenopathy, and hepatomegaly. WBC count in the peripheral blood was 22.5 x 109/l, and the smear revealed the presence of pleomorphic lymphoid cells, mainly medium sized, with irregular nucleus or nuclear notches. Immunophenotype studies of mononuclear cells of the peripheral blood showed characteristic membranous phenotype for Mantle zone lymphoma in leukaemic phase: Smlg+ (lambda light chain); HLA-DR+; CD19+; CD22+; CD5+; CD10-; CD25-. Pro-MACE-Cyta-bom protocol was applied resulting in a 13-month-lasting remission. The total survival was 20 months, suggesting poor prognosis of leukaemic phase of Mantle zone lymphoma.
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PMID:[Leukaemic phase of Mantle zone (intermediate) lymphoma--case report--]. 1797 26

We present the case of a 53 years old woman diagnosed with splenic marginal zone lymphomas with plasmacytic differentiation (after a lymph node biopsy), who, complained of mild asthenia, weight loss (about 10 kg in 9 months), spatial disorientation during the last period. The clinical examination revealed slight pallor, normal cardiovascular and respiratory examination; painful cervical, about 5cm in diameter and also non-painful inguinal lymphadenopathies, increased consistency, freely movable, about 2 cm in diameter. The patient presented enlarged liver (lower limit at 3 cm below the ribs) and spleen (inferior pole at the ombilicus). The laboratory tests showed leucocytosis with lymphocytosis-a clonal population of lymphocytes- CD19+, CD20low+, CD22+, CD5low+, CD24+, CD200low+, CD79B+, CD43-, FMC7+/-, CD10+/-, CD34-, BCL2+, TdT-, CD34-, CD10-, CD3-. We suggested the diagnosis of mantle cell lymphoma, blastoid variant and performed a bone marrow biopsy . The bone marrow biopsy excluded the diagnosis of mantel cell lymphoma, based on the absence of cycline D1. The histopathological appearance and the immunohistochemical tests (CD20+, CD79a+, CD5low+, TdT-, CD34-cycline D1-) suggested a blastoid variant of small lymphocytic lymphoma.
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PMID:Difficulty in classifying a B cell chronic lymphoproliferative disorder CD5+. 2361 Sep 78