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)

Two cases of T-cell chronic lymphoid leukaemia in adults were classified by recently developed immunological methods. Clinically there was splenomegaly, hepatomegaly, marked anaemia, para-aortic lymph nodes, cutaneous infiltration, little peripheral lymph-node enlargement and significant marrow infiltration. Immunological tests of membrane antigens revealed T-cell determinants. T-CLL may differ in its course and prognosis from B-CLL and may require different treatment.
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PMID:[T-cell leukaemia in adults: report of two cases (author's transl)]. 30 81

A 48-year-old male was admitted to our hospital on April 20, 1989 because of general fatigue and abdominal fullness. Physical examination showed hepatomegaly, massive splenomegaly, and systemic lymphadenopathy. Hematological findings revealed WBC 73,000/microliters, RBC 289 x 10(4)/microliters, Hb 8.0g/dl, and platelet 9.1 x 10(4)/microliters. WBC differential count demonstrated a mixture of 63% matured small lymphocytes and 32% prolymphocytoid cells. Bone marrow aspiration was unsuccessful with a dry tap. Surface marker analysis of peripheral blood lymphoid cells disclosed that they were positive for anti-HLA-DR, CD 5, CD 19, CD 20, CD 21, CD 25, Sm-IgM, Sm-IgD, and Sm-K. He was diagnosed as B-CLL/PL, and treated with VEPA with partial remission. CLL/PL which was advocated by Melo in 1986 is regarded as a distinct clinical entity intermediate between CLL and PLL in clinical and laboratory features. Our case is interesting with regard to good response to combination chemotherapy, though most cases of CLL/PL have a resistance to standard chemotherapy.
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PMID:[B-chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL)--a case report]. 228 70

T-cell chronic lymphocytic leukemia (T-CLL) accounts for about 2% of the various types of CLL and can be subtyped into helper/inducer (h/i) and cytotoxic/suppressor (c/s) cell membrane phenotypes. Seven patients with CLL were shown to have T-CLL with a h/i cell membrane phenotype; four with monoclonal antibody reagents and three by demonstration of the E-rosette receptor and focal acid alpha naphthyl acetate esterase activity. The clinical courses, treatment responses, and laboratory findings of these seven patients were reviewed to determine the prognosis and unique clinicopathologic features of this subtype. Two patients presented with skin rashes, and five were diagnosed during evaluation for other medical problems. Initially, four patients had splenomegaly and two had lymphadenopathy, but none of the patients had hepatomegaly. Morphologic examination revealed uniform, small lymphocytes in three patients, and the lymphocytes had nuclear indentations in four patients. Sera from the three patients tested were negative for antibody to the human T-cell leukemia/lymphoma virus I. Peripheral blood mononuclear cells from one patient showed normal interleukin-2 production and lacked antibody-dependent cell-mediated cellular cytotoxicity and natural killer activity. Cytogenetic analysis was done on one patient, revealing an abnormal clone with several chromosomal abnormalities, including an X;14 translocation with a break point at 14q11. All patients required chemotherapy, and all died a median of 21 months from the time of diagnosis. The findings in these patients, in addition to those in 31 patients described in the literature, indicate that h/i T-CLL is associated with a poor prognosis and has distinct clinical and pathologic features that separate it from c/s T-CLL, adult T-cell leukemia/lymphoma, the cutaneous T-cell lymphomas, and B-CLL.
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PMID:T-cell chronic lymphocytic leukemia with a helper/inducer membrane phenotype: a distinct clinicopathologic subtype with a poor prognosis. 293 74

The serum levels of soluble intercellular adhesion molecule 1 (ICAM-1) were significantly elevated (P < .001) in patients with chronic B-lymphocytic leukemia (B-CLL, n = 113) compared with healthy controls (n = 31). sICAM-1 levels in B-CLL were positively correlated to the tumor mass as reflected by the modified Rai and the Binet staging systems, lymphocyte counts, and isolated spleno/hepatomegaly. During disease progression or regression on cytoreductive therapy, the circulating sICAM-1 levels changed accordingly. sICAM-1 was also correlated to a kinetic parameter such as the lymphocyte doubling time. Furthermore, the serum sICAM-1 levels were inversely correlated to hemoglobin levels in patients with early clinical stage, and this may turn out to be of prognostic value. sICAM-1 was compared with other serum markers said to reflect disease activity in B-CLL, ie, soluble CD23, thymidine kinase, lactate dehydrogenase (LDH), and beta 2-microglobulin. sICAM-1 was equally well or better correlated to clinical stage and lymphocyte doubling time. In univariate regression analysis, all serum markers but LDH correlated with survival, and in multivariate analysis, sICAM-1 was the only marker approaching significance for additional prognostic information when included after clinical stage and lymphocyte doubling time. Based on the present observations, it appears that prospective studies repeatedly monitoring serum sICAM-1 in B-CLL are justified.
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PMID:Serum levels of soluble intercellular adhesion molecule 1 are increased in chronic B-lymphocytic leukemia and correlate with clinical stage and prognostic markers. 777 31

A 59-year-old man was admitted to our hospital on May 17, 1991 because of dizziness and a sense of abdominal fullness. Physical examination on admission showed splenomegaly without hepatomegaly or lymphadenopathy, and blood examination revealed normocytic anemia, thrombocytopenia and marked leukocytosis of 16,800/microliters with 87% lymphoid cells. Prolymphocytoid cells formed 28% of the lymphoid cells. Bone marrow aspiration revealed massive infiltration of lymphoid cells. Surface marker analysis showed that the lymphoid cells were positive for anti-HLA-DR, CD 5, CD19, CD20, CD21, SmIgM and SmIgD. The patient was diagnosed as having B-CLL/PL, according to the classification advocated by Melo in 1986, and initially treated with vindesine + prednisolone + pirarubicin (VP-THP). However, the prolymphocyte count increased, so we changed to VP-THP + cyclophosphamide (VEP-THP), and remission was obtained. CLL/PL is a rare disease in Japan but we obtained a good response to chemotherapy.
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PMID:[A case of B-chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL)]. 842 81

The incidence of trisomy 12 and p53 deletion was studied in a group of chronic B-lymphocytic leukemia (B-CLL) patients, using fluorescence in situ hybridization (FISH). Trisomy 12 was detected in eight of 50 patients (16%) and p53 deletion in six of 38 cases analyzed (15.8%). A statistically significant difference was observed between the incidence of trisomy 12 in patients with typical and atypical morphology (3.03% versus 41.18%). No correlation was found between this alteration and the rest of the clinical and biological parameters studied (adenopathies, hepatomegaly, splenomegaly, lymphocyte count, staging, CD11c expression, and resistance to chemotherapy). The p53 deletion was correlated with the presence of hepatomegaly and splenomegaly, advanced stage of disease, and resistance to conventional chemotherapy. The application of FISH to whole blood cell nuclei, without prior manipulation or culture, showed a higher percentage of cells with trisomy 12 than when the method was used following culture. We conclude that 1) FISH is a simple and sensitive technique for the detection of numerical and structural chromosome abnormalities; 2) Its application to uncultured samples obviates the alteration of results originated by the probable growth advantage of the normal or neoplastic cell population in vitro; 3) Trisomy 12 appears to define a B-CLL subgroup of atypical morphology; and 4) The p53 deletion is correlated with advanced stage of disease and resistance to treatment.
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PMID:Trisomy 12 and p53 deletion in chronic lymphocytic leukemia detected by fluorescence in situ hybridization: association with morphology and resistance to conventional chemotherapy. 883 Jul 19

In Taiwan, as in other areas of Asia, the incidence of chronic lymphocytic leukemia (CLL) is low. A retrospective analysis was conducted to elucidate the clinicopathologic features of CLL patients in Taiwan. Of the 47 cases of CLL enrolled in this study, 45 were immunophenotyped as B-CLL; the other 2 were T-CLL. It was found that the lower the Binet and Rai stages of the B-CLL, the longer patients survive (p = 0.0131 and 0.0142, respectively). Univariate analysis showed that fatigue, splenomegaly, hepatomegaly and anemia are associated with poor survival with p values of 0.0203, 0.0184, 0.0001 and 0.171, respectively. By multivariate analysis with Cox's proportional hazard model, hepatomegaly and decrease in body weight were the two most significant predictors of survival. However, molecular parameters of kappa or lambda immunoglobulin (Ig) gene rearrangement or double allele rearrangement of Ig gene did not significantly increase the predictability of the prognosis.
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PMID:Genotypic characterization and multivariate survival analysis of chronic lymphocytic leukemia in Taiwan. 915 61

A 61-year-old man was found to have mild lymphocytosis during a medical checkup and was referred to our hospital in 1993. Physical examination showed a mild hepatomegaly, and bone marrow examination revealed the proliferation of monoclonal mature B cells. He was diagnosed with B-CLL. Chlorambucil and then fludarabine were effective initially. However, large transformed cells started to increase in 2000, and the increment became uncontrollable and platelet transfusion dependence developed. He was admitted to our hospital in October 2001. He intermittently received a total of 32 administrations of rituximab. The total dose was 15, 500 mg. The toxicity was tolerable and he became transfusion independent for 6 months. However, systemic lymphoadenopathy and hepatomegaly did not respond to the drug. We conclude that rituximab treatment is useful for refractory B-CLL because of its excellent safety.
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PMID:[Drug-resistant B cell chronic lymphocytic leukemia safely treated with rituximab to control leukemic cell increment and platelet transfusion dependence]. 1499 66