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)

Three children developed acute veno-occlusive disease of the liver following combination chemotherapy for acute myelocytic leukemia. The clinical presentation was similar in all three, with acute onset of hepatomegaly and thrombocytopenia in the absence of significant transaminasemia or icterus. In all three patients, radionuclide imaging with technetium-99m sulfur colloid showed hepatosplenomegaly, decreased liver uptake, and increased splenic activity. The results of liver biopsy established the diagnosis, revealing marked centrilobular congestion with hemorrhage into the spaces of Disse, atrophy of central hepatic cords, and edema of the walls of the central and sublobular veins. Each patient showed marked improvement following temporary cessation of chemotherapy. The diagnosis of veno-occlusive disease is suggested by the triad of: (1) clinical signs and symptoms; (2) scintigraphic findings; and (3) temporal relationship to chemotherapy.
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PMID:Veno-occlusive disease of the liver in children following chemotherapy for acute myelocytic leukemia. 657 67

We describe 3 cases of acute graft-versus-host (GVH) disease in patients with acute myeloid leukaemia following transfusions taken from non-HLA-identical healthy donors. The leucocyte transfusions were given because of severe bone marrow aplasia and granulocytopenia following leukaemia induction treatment. The first patient had an acute GVH reaction with an erythrodermia-like skin reaction all over and associated with severe abdominal cramping, enlarged liver and pathological liver function tests. The second patient had a relatively mild skin reaction and enlarged liver. Both died of severe pulmonary infection. The third patient also had a mild skin reaction and enlarged liver. He died of pulmonary embolism. The diagnosis of GVH of the latter 2 cases was made on skin biopsy. The autopsy samples revealed in all cases a heavy lymphocytic infiltration of the kidneys and liver portal area. Until more precise guidelines can be established, irradiation of blood cell products given to patients with neutropenia due to leukaemia induction treatment should be considered.
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PMID:Graft-versus-host reaction in 3 adult leukaemia patients after transfusion of blood cell products. 658 Jul 18

Fifty-two patients with different types of leukemia [27 chronic lymphocytic leukemia (CLL), 10 acute myeloblastic leukemia (AML), eight acute lymphoblastic leukemia (ALL), four chronic myelogenous leukemia (CML), and three hairy cell leukemia (HCL)] had abdominal and pelvic CT. The radiographic features are reviewed to evaluate the role of CT in diagnosis and management of these patients. The highest incidence of lymphadenopathy was observed in CLL followed by HCL and ALL. Other findings included splenomegaly, hepatomegaly, ascites, pleural effusion, microabscesses in liver and kidney, and visceral involvement, i.e. intestine, bladder, and bone.
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PMID:Abdominal and pelvic computed tomography in leukemic patients. 659 May 76

Recently, several specific chromosomal abnormalities have been associated with distinctive clinical and/or morphological subtypes of acute nonlymphocytic leukemia (ANLL). To further investigate the clinical utility of karyotype analysis in ANLL, we have examined G-banded metaphase chromosomes at diagnosis in 61 consecutive patients. Of the 60 patients who had adequate mitoses, 47 (78%) had a clonal chromosome abnormality. The sole karyotypic abnormality found in 5 patients was a del(16)(q22). The unique pretreatment characteristic of these 5 patients was marrow eosinophilia ranging from 8% to 54%. No other patient had more than 4% marrow eosinophils. Among the patients with eosinophilia, all had Auer rods, serum muramidase was elevated in the 4 tested, and 4 had hepatomegaly at presentation. Both patients who survived initial treatment remain in complete remission at 23+ and 33+ mo. The data suggest that we have identified a new cytogenetic-clinical subtype of ANLL defined by the del(16)(q22).
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PMID:Partial deletion of the long arm of chromosome 16 and bone marrow eosinophilia in acute nonlymphocytic leukemia: a new association. 683 Oct 56

The occurrence of a transitory aplasia followed shortly thereafter by an acute leukemia in a known sequence in children is rare. We report 13 observations involving children between 6 months and 10 years of age. Splenomegaly was observed in 5 patients and hepatomegaly in two. There was a tricytopenia in 5 cases, bicytopenia in 4 and an isolated cytopenia in the remaining 4 cases. The phase of aplasia was short, lasting from 6 to 30 days. Complete bone marrow recovery occurred with integral restitution. Remission was spontaneous or followed transfusion or corticosteroid therapy and lasted for 2 to 6 months. The leukemia had no particular character when it appeared: there was one acute myeloblastic leukemia, 11 acute lymphoblastic cases and one sarcoma. Median survival time was 5 to 32 months and relapses were not aplasic. It is noteworthy that among these cases there are two long remissions lasting more than ten years. The interpretation of these observations is difficult because of the following choice: an initially non-leukemic aplasia or a leukemia present at the onset but undetected, camouflaged or confined to several infrequent blast cell islets.
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PMID:[Acute curable preleukemic bone marrow aplasia in children]. 712 79

Forty patients (9 females and 31 males; mean age 41.9 years) with CD7+ acute myelocytic leukemia (AML) were investigated; they were classified into the following subgroups according to French-American-British classification: 15 M1, 18 M2, 3 M4, and 4 M5. Leukemic cells from all the patients were negative for T-cell-specific antigens, surface CD3, and T-cell-receptor molecules. The sex and age distributions were different from those of CD7- AML patients (P < .01). Hepatomegaly and central nervous system involvement were also frequent in the CD7+ AML patients. The phenotype of and responsiveness to hematopoietic growth factors by the leukemic cells showed their immaturity, as evidenced by frequent expression of CD34, HLA-DR, and TdT, and the greatest growth response to interleukin-3. No particular karyotypic abnormality was shown. One hundred eighty AML patients were treated with a therapeutic regimen routinely used for AML. The CD7+ AML patients showed a significantly lower response than CD7- AML patients (P < .01), and had a poorer prognosis (P < .01). CD7+ AML patients with M1 or M5b had unfavorable responses to the therapeutic regimen in comparison with patients with M2, M4, or M5a. In addition, 3 of 4 CD7+ CD2+ AML patients, who did not respond to the therapy, were induced into complete remission with an acute lymphoblastic leukemia therapy. The results presented here indicate the diagnostic importance of CD7 positivity in AML, suggesting that the cellular and clinical characteristics of CD7+ AML are sufficient for it to be recognized as a distinct category of AML.
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PMID:Clinical importance of CD7 expression in acute myelocytic leukemia. The Japan Cooperative Group of Leukemia/Lymphoma. 769 52

The French-American and British (FAB) classification of 62 consecutive cases of acute myeloid leukemia was undertaken. AML-M2 was the commonest FAB type (32.26%), followed by M1 and M4 (22.58% each), M5 (8.6%) and M6 and M7 (1.61% each), respectively. One of the patients was diagnosed as AML-MO (not a FAB type). The mean age of M1, M2, M3 and M5 cases was between 25 and 29 years, whereas in M4 patients it was 45.6 years. AML-M2, M4 and M5 were commoner in males, M1 in females and M3 equal in both sexes. Feeling of weakness, easy fatiguability and pallor were invariably present in all FAB types. All the patients of M1 and M5, 85% of M2, 64% of M4 and 50% of M3 presented with fever. Bleeding manifestations were most frequent in M3 cases followed by M5, M1, M4 and M2, respectively. Hepatomegaly and splenomegaly were relatively less prominent features in M3 as compared to other FAB types. Amongst the haematological parameters, anaemia was more severe in M1, leucocytosis in M2 and thrombocytopenia in M3 cases as compared to other FAB types.
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PMID:Acute myeloid leukemia-FAB classification and its correlation with clinico-haematological features. 811 48

With an increasing number of elderly patients presenting with acute myeloid leukaemia and with recent reports of worthwhile remission rates and survival times following treatment with intensive chemotherapy, there is a pressing need to identify criteria to assist in the selection of appropriate therapy for elderly patients. We have performed multivariate Cox proportional hazard regression analysis of data prospectively collected during the treatment of 104 patients aged 60 and over treated in a multi-centre study with a standardized regimen of mitozantrone and cytosine arabinoside for induction and consolidation. Four readily available parameters, namely urea, performance status, peripheral blood blast count and presence of hepatomegaly, were identified from which a prognostic model to predict survival has been developed. The model was found to be accurate in predicting survival in the cohort of patients from which it was developed, but needs to be validated in a further test population studied prospectively. Its simplicity suggests that it may be particularly useful in the selection of elderly patients with AML most likely to benefit from intensive chemotherapy.
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PMID:Prognostic factors in elderly patients with acute myeloid leukaemia: development of a model to predict survival. 828 Jun 3

The objective of this study was to evaluate the treatment outcome of children with acute leukemias at a university hospital in Singapore. Between January 1988 and January 1994, 66 children were treated, comprising 13 cases of acute myeloid leukemia (AML) and 53 acute lymphoblastic leukemia (ALL). The 2-year disease-free survival (DFS) was computed according to the Kaplan-Meier method. The results showed that the survival of AML was poor, with a 2-year DFS of only 30%. The major cause of death for AML was leukemia and leukemia-related complications, such as hemorrhage and severe infections. In contrast, a 62% 2-year DFS was achieved for ALL. It was found that marked hepatosplenomegaly (enlarged liver and/or spleen > or = 10 cm below the costal margin) at presentation correlated with a significance shortened survival in our patients with ALL. The major cause for treatment failure in ALL was recurrence of disease. We conclude that the DFS for our patients with ALL at 2 years was fair. The treatment results for AML were poor, but the numbers are too small to make any definite conclusions.
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PMID:Survival of childhood leukemia in Singapore. 861 64

Eighty six of 430 acute myeloblastic leukemia (AML) patients (20.0%) and forty of 173 acute lymphoblastic leukemia (ALL) patients (23.1%) had CD7 on their leukemia cells. CD7(+) AML occurred at a younger age than CD7(-) AML, and is more frequent in males. Hepatomegaly and central nervous system involvement were also more frequent in CD7(+) AML than in CD7(-) AML. The age of onset of CD7(+) ALL is also younger than that of CD7(-) ALL. Phenotypically, CD(+) AML expressed CD34, HLA-DR, and TdT more frequently than CD7(-) AML while CD7(+) ALL expressed CD13/33 more often than CD7(-) ALL cells responded most significantly to interleukin 3 (IL-3), whereas most CD7(-) AML cells responded more significantly to granulocyte macrophage-colony stimulating factor (GM-CSF) and/or granulocyte (G)-CSF than to IL-3. CD7(+)sCD3(-)CD4(-)CD8(-) ALL expressed G-CSF receptor and c-kit mRNA more frequently, which is not usual in other types of ALL. P-glycoprotein (P-gp)/multi-drug resistance gene (MDR1), thought to be expressed in hematopoietic stem cells, is expressed in CD7(+) AML and CD7(+)sCD3(-) CD4(-)CD8(-) ALL significantly more often than in CD7(-) acute leukemias and the CR rate and overall survival of CD7(+)AML was worse than CD7(-) AML. These data, collectively, suggest the close association of CD7(+) AML and CD7(+)sCD3(-)CD4(-)CD8(-) ALL, not only the common expression of CD7 itself but also because their phenotypical immaturity, cytokine receptor expression, P-gp/MDR1 expression and clinical manifestations including the frequent occurrence in males and the poor prognosis. We propose that CD7(+) acute leukemia is an hematopoietic stem cell leukemia which may be separate entity.
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PMID:Biological characteristics of CD7(+) acute leukemia. 872 5


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