Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented with markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic, autoimmune and metabolic liver diseases were excluded. Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease. Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen. Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+, CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, thrombocytopenic purpura and blast in the peripheral blood. In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic T-cell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.
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PMID:Hepato-splenic lymphoma: a rare entity mimicking acute hepatitis: a case report. 1280 Feb 62

The prognostic significance of immunophenotypical properties of leukaemic cells is well known. However, the biological and clinical significance of CD7 and CD56 antigen expression in acute leukaemias are not clearly established. In patients with acute leukaemias, we identified CD7 and CD56 expression and analysed their associations with markers expressed early in haemopoietic ontogeny and clinical parameters. Among 22 patients with acute leukaemia [12 acute myeloblastic leukaemia (AML), 10 acute lymphoblastic leukaemia (ALL)], we found CD7 positivity in 15 of 22 patients (68%) and CD56 positivity in four patients (18%). CD7 positivity was observed in seven patients (58%) with AML and in eight patients (80%) with ALL. CD56 positivity was observed in three patients (25%) with AML and one patient (10%) with ALL. Lymphadenopathy was present in five patients and associated with hepatosplenomegaly in three patients with ALL. Splenomegaly and hepatomegaly were present in three patients with AML. Central nervous system involvement was seen in one patient with ALL. Complete remission was achieved in nine patients (41%) (five ALL and four AML). Our data showed that CD7 and CD56 positivity at diagnosis associated with low remission rate and biological aggressiveness in a significant proportion of patients. We suggest the evaluation of CD7 and CD56 in all patients with acute leukaemias at the time of diagnosis in view of poor clinical outcome.
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PMID:The importance of CD7 and CD56 antigens in acute leukaemias. 1505 63