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)

Chronic lymphocytic leukaemia was diagnosed in 1957 in a 29-year-old male patient who lived 31 years after that time. At the time of diagnosis he was in good general condition with the enlarged liver (4 cm) and spleen (5 cm). In peripheral blood the leukocyte count was 47 x 10(9)/1 with 80% lymphocytes (38 x 10(9)/1), Hb 106 g/1; the bone marrow showed hypercellularity with predominance of lymphocytes. Treatment with corticosteroids was during 1958-1966. In 1967 Chlorambucil was given for 3 months, in 1972 for one month, inducing normalization of leukocyte count, 5.7 x 10(9)/1 with 40-50% of lymphocytes (3 x 10(9)/1). In 1977 the bone marrow showed slight lymphocytosis (38%). Numerous infections were recorded. Splenomegaly disappeared in 1980. In 1982 macrocytic anaemia was observed. Diagnosis of pernicious anaemia was established based on megaloblastic bone marrow and positive Schilling test. Vitamin B12 treatment resulted in reticulocyte response (crisis) and normalization of haemoglobin level. In 1988 light anaemia and melena appeared. Gastroscopy revealed lesions in the fornix histologically identified as ventricular carcinoma. It can be presumed that the appearance of pernicious anaemia as an autoimmune disease is analogous to other autoimmune complications in chronic lymphocytic leukaemia (haemolytic anaemia, thrombocytopenia).
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PMID:[Chronic lymphocytic leukemia complicated by pernicious anemia during long-term remission]. 213 9

We report 26 elderly patients (median age 68.3 years) who met diagnostic criteria for B-cell chronic lymphocytic leukaemia (B-CLL) but whose lymphocytes lacked CD5 expression. Haematological and clinical features of this CD5- series were compared with those of 333 CD5+ B-CLL patients from the same institute. No significant differences were observed regarding peripheral blood (PB) and bone marrow (BM) lymphocytosis, Hb level, platelet count, incidence of adenomegaly, hepatomegaly or splenomegaly or diffuse BM pattern. Due to an absence of nodal enlargements or to general clinical condition, lymph node biopsy was performed in only three patients, while spleen histology was examined in two cases following splenectomy. All histological results confirmed the clinical diagnosis of CLL. The distribution of the CD5- subjects according to the different staging categories proposed by Rai, Binet and Mandelli was similar to that of CD5+ subjects. Ten patients received standard chemotherapy with Chlorambucil (CHL) and Prednisone (PDN). All achieved partial remission, although one of these patients later died of disease progression; 80 months after diagnosis. We conclude that rare cases of CD5- lymphocytosis fulfilling all criteria for B-CLL may occur. Haematological and clinical features at presentation and the response to conventional treatment with Chlorambucil support our hypothesis of considering this disease as a less frequent subgroup of B-CLL.
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PMID:CD5 negative lymphocytosis mimicking typical B-chronic lymphocytic leukaemia. Description of 26 cases. 769 97

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