Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between 1979 and 1987, 82 adults (age 14-71 years) with acute lymphoblastic leukaemia (ALL) were treated with a 6-course protocol called HEAVD, the main feature of which was the early postremission administration of escalating doses of doxorubicin (total 405 mg/m2) and cyclophosphamide (total 2.5 g/m2). A complete remission (CR) was attained in 66 patients (80%, 95% confidence intervals, [CI] 71%-89%). Factors affecting favourable CR achievement were age less than 60 years and absence of lymphadenopathy-hepatosplenomegaly at presentation (P less than 0.05). Median duration of CR was 27 months. 26 patients remain in first continuous and unmaintained CR, 18 of whom between 5.9 and 11.1 years, for an estimated 39% prolonged disease-free survival (95% CI 27%-51%). CR duration correlated significantly with absolute blast cell count (15 x 10(9)/l or less compared to more) and age (30 years or under compared to over). Overall, 29 patients are alive with a median follow-up of 6.7 years, the projected long term survival being 35% at 11 years (95% CI 24%-46%). Treatment-related toxicity included 1 lethal case of L-asparaginase-related thromboembolism and 3 toxic deaths among 66 CR patients. Late-onset toxicity was not observed in long-term survivors. The relatively late occurrence of endpoint events (relapse and death) in adult ALL confirms that long-term updating is necessary to determine the curative potential of modern chemotherapy programs for the disease.
 ...
PMID:Long-term results of the HEAVD protocol for adult acute lymphoblastic leukaemia. 182 18

To determine the chromosomal abnormalities and their clinical significance in adult acute lymphoblastic leukemia, karyotypic analyses with R- and/or G-banding were performed in 101 newly diagnosed ALL patients. The results showed that 69 (68.32%) cases had clonal chromosomal abnormalities and specific abnormalities including Philadelphia chromosome (32.67%), t(4;11) (2.97%), t(11;14) (3.96%), abnormalities of 8q24 (2.97%), 6q- (4.96%). Patients with Ph chromosome showed higher leukocyte counts and more frequent hepatosplenomegaly and most of them were L2 and B lineage subtypes. They had lower complete remission (CR) rate, needed longer time to achieve CR and relapsed earlier as compared with patients without Ph chromosome. These suggested that chromosomal abnormalities are very frequent and some specific abnormalities had specific clinical features.
 ...
PMID:[Chromosomal abnormalities and their clinical significance in acute lymphoblastic leukemia in adults]. 1037 15

Patients suffering from adult acute lymphoblastic leukemia are acutely ill and present most commonly with fever, pallor, bleeding, lymphadenopathy, hepatosplenomegaly and presence of lymphoblasts in the peripheral blood and bone marrow. We describe a rare presentation of acute lymphoblastic leukemia, in a young adult male who had vague and minimal symptoms with mild splenomegaly. There was severe eosinophilia along with absence of blasts in the peripheral blood, and 40% blasts with increase in eosinophils in the bone marrow. The blasts were positive for common precursor B cell markers on flow cytometry. The patient had a unique cytogenetic abnormality t(7;12)(q22;p13),-9, not previously described in acute lymphoblastic leukemia. He was categorized as poor risk due to failure to achieve complete remission after induction with UK ALL XII chemotherapy.
 ...
PMID:Adult B lymphoblastic leukaemia/lymphoma with hypodiploidy (-9) and a novel chromosomal translocation t(7;12)(q22;p13) presenting with severe eosinophilia - case report and review of literature. 1954 91

Infiltration of the liver with consecutive severe dysfunction is rarely seen in adult acute lymphoblastic leukemia (ALL). We describe a case of a 32-year-old woman with severe icterus, thrombocytopenia, neutropenia and hepatosplenomegaly. ALL was diagnosed. One day after admission, she developed hepatic encephalopathy with ammonia concentrations in plasma >100 micromol/l. Hepatic infiltration was presumed and chemotherapy was initiated immediately which led to resolution of hepatic encephalopathy and complete hematological remission. Clinicians should be aware of unusual presentations of ALL and difficulties for the application of chemotherapy in patients with liver failure.
 ...
PMID:Successful treatment of hepatic encephalopathy in a patient with acute lymphoblastic leukemia. 1988 78