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Query: UMLS:C0023467 (acute myeloid leukemia)
 35,200 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired enzymatic activity defects of erythrocyte pyruvate kinase, glucose phosphate isomerase and phosphofructokinase have been studied in patients with acute myeloid leukemias, sideroblastic refractory anemias and unclassified acquired dyserythropoiesis. 6 patients with acute myeloid leukemia had a lowered erythrocyte pyruvate kinase activity; in 5 of them the concentration of the "pyruvate kinase"-antigen was parallely decreased, in such a manner that the ratio enzyme activity/immunologic reactivity (i.e. the molecular specific activity) was normal. In 1 patient with acute leukemia, 4 with refractory anemia and 1 with acquired dyserythropoiesis the defect of the pyruvate kinase activity was associated with a normal antigen concentration (and, therefore, the molecular specific activity in whole hemolysate was lowered). The enzyme activity was restored by incubation with SH reagents in two cases and by partial purification as often as it was performed. The electrofocusing pattern of erythrocyte pyruvate kinase was normal in both these types of defects. In two patients with so-called "acquired dyserythropoiesis" an erythrocyte glucose phosphate isomerase deficiency has been detected; in both the cases it was associated with a parallel decrease of the antigen concentration. The residual enzyme had a normal electrofocusing and electrophoretic pattern and a normal heat stability; the enzyme activity could not be restored by any treatment. In 1 patient with erythroleukemia and in 1 other with acquired dyserythropoiesis the erythrocyte phosphofructokinase activity was lowered. The enzyme activity was not restored by cross incubation in isologous plasma or by the SH reagents. In one case immunologic study could be performed, indicating that the enzyme defect was mainly due to the decreased ratio of the muscle type subunit of the erythrocyte phosphofructokinase. The electrofocusing pattern of deficient phosphofructokinases was normal. Finally, we point out the probable existence of several direct mechanisms, genetic and post translational, accounting for the acquired enzyme defects of red blood cells in various blood disorders.
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PMID:Mechanisms of the acquired erythrocyte enzyme deficiencies in blood diseases. 13 55

This report deals with a new syndrome characterized by refractory anemia, an aberration of chromosome 5 (deletion of the long arm) and the subsequent appearance of acute myeloid leukemia. The results stress the importance of chromosomal studies in cases of unclear, long-lasting and therapy-resistant anemia, since they may allow the early recognition of the development of acute leukemia.
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PMID:[Acute myeloid leukemia with chromosome 5 deletion (a new syndrome)]. 29 13

The case of a 4-year-old boy with ALL, possibly developing subsequent to a lymphoma involving the thoracic cavity, which was shown to be of the T-cell type, is presented. The leukemic cells had a 5q-- anomaly, which had previously been described only in cases of refractory anemia and/or AML. The 5q-- abnormality was invariably accompanied by a 9p-- chromosome in the leukemic cells. The interstitial deletion leading to the 5q-- chromosome was shown with banding techniques to be similar to that described previously in myeloproliferative disorders. Some aspects of the 5q-- anomaly in ALL and its relation to previous experience with this karyotypic change are discussed.
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PMID:Chromosomes and causation of human cancer and leukemia. XXXIII. 5q-- in a case of acute lymphoblastic leukemia (ALL). 31 53

Approaches to the diagnosis and classification of preleukemic states involving chronic cytopenias are presented and discussed. Diagnosis of these states is facilitated by the identification of anomalies in all the myeloid cell lines (i.e., those derived from the bone marrow). These cellular anomalies may be morphologic, biochemical, or functional in nature or may affect the quantity of cell in each line in the bone marrow and the peripheral blood. Such anomalies may occur alone or may be associated. A tentative classifiction is proposed which is based on one or several of these anomalies. Among the quantitative criteria of classification is a moderate and static excess of myeloblasts and promyelocytes in the bone marrow. Refractory anemia with an excess of myeloblasts (RAEM) in the most frequent of these states. Its main clinical and hematologic features are described. The disease course is quite typical, the mean survival being 20 months; some patients survive for more than 30 months. Acute myeloid leukemia (AML) was the cause of death in less than 28% of cases. Infection in the absence of severe neutropenia was frequent. The relationship between RAEM and AML is disc,ssed, and the individual characteristics of RAEM are emphasized.
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PMID:Preleukemic states. I. Definition and classification. II. Refractory anemia with an excess of myeloblasts in the bone marrow (smoldering acute leukemia). 100 6

Seventy-nine patients with a refractory anemia and partial myeloblastic medullary infiltration have been studied, according to a prospective common protocol. All the patients have been treated with androgens, at high dosage and for at least 10 months if surviving. This study enables to precise the natural history of the disease and to define some criteria valuable for the prognosis. It demonstrates that the classification of this clinical entity as a smoldering or pre-leukemia is justified: 63% of the patients died from acute myeloblastic leukemia. The disease is very severe: the median of survival from the diagnosis is only 13 months. Androgen therapy appears to have little if any effect on the anemia, granulocytopenia and thrombocytopenia; it does not seem to increase the patients' life expectancy.
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PMID:[Refractory anemias with partial myeloblastosis. Analysis of a protocol comprising 79 cases. 1. Clinical characteristics and evolution under androgen therapy]. 106 63

We report a case of essential thrombocythemia (ET) that climaxed in acute myeloid leukemia after developing into refractory anemia. The male patient had ET that was stable for 8 years on carboquone therapy. However, at the age of 72 years he developed an acute terminal illness that was characterized by severe pancytopenia, circulating myeloblasts, extensive bone marrow infiltration by myeloblasts, and an abnormal karyotype [46, XY, t(8q-; 20q+)]. He subsequently died of severe bilateral pneumonia and heart failure. This case suggests that ET may be similar to polycythemia vera; progression to leukemia is unusual except after chemotherapy. Therefore, treatment of patients with asymptomatic ET may not be advisable.
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PMID:Essential thrombocythemia developing into refractory anemia and complicated by acute myeloid leukemia. 128 33

Refractory anemia (RA) is the only myelodysplastic syndrome (MDS) devoid of quantitative marrow diagnostic criteria. The diagnosis rests mainly on the subjective identification of qualitative abnormalities according to the French-American-British criteria (FAB) involving one or more bone marrow hematopoietic cell lineages. The occurrence of nonrandom chromosome abnormalities remains the hallmark of the disease and the only means of investigation which confirms the disease objectively. With the purpose in mind to further characterize RA among MDS, we have undertaken a prospective high resolution banding chromosome analyses of bone marrow cells in patients with primary refractory anemia (PRA) with the aim of defining a cytogenetic phenotype and of assessing the clinical relevance of clonal abnormalities at initial diagnosis. Of 39 patients consecutively referred for chromosome analyses with a diagnosis of RA according to the FAB criteria, 27 patients had PRA and fulfilled our criteria for adequate chromosome analyses. Median age was 68 years. Fourteen of 27 patients (52%) had clonal chromosomal abnormalities at diagnosis. None of the patients showed a complex karyotype; 9/14 (64%) had a mixture of normal and abnormal cells. Interstitial or terminal deletions, involving chromosomes 5, 6, 7, 9, 11, 12, and 20, were found in 11/14 (79%) of the patients. Comparison of survival between patients with and without abnormalities showed no difference. The presence of clonal abnormalities did not predict transformation to acute myeloblastic leukemia (AML) nor was it associated with poor survival. In this study, patients with PRA were found to have a predominant pseudodiploid karyotypic pattern characterized by interstitial and/or terminal deletions as opposed to derivatives, specific and non-specific balanced translocations, or other structural and numerical abnormalities. We were unable to reveal any prognostic significance to the presence of these clonal abnormalities at initial diagnosis.
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PMID:Cytogenic characterization of primary refractory anemia. 128 85

A 56-year-old African-American man presented with fever of unknown origin and peripheral blood and bone marrow findings of myelodysplastic syndrome (MDS): refractory anemia with an excess of blasts in transformation that subsequently progressed to acute myeloblastic leukemia (AML). Ultrastructural study of two bone marrow specimens having the findings of MDS revealed frequent, large tubuloreticular structures (TRS) in lymphocytes, plasma cells, macrophages, and endothelial cells. Several cylindrical confronting cisternae (CCC) were present in macrophages and an endothelial cell. Two partially developed CCC were present in a plasma cell. TRS and CCC were not observed in eight subsequent bone marrow specimens obtained during the 9-month course of the AML. This is the first reported occurrence of TRS and CCC in MDS. These inclusions are probably related to an unidentified viral infection or possibly to cytokines released by the dysplastic hematopoietic cells.
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PMID:Cytomembranous inclusions in myelodysplastic syndrome. 133 38

At transformation of refractory anemia with ring sideroblasts to acute nonlymphocytic leukemia (ANLL) the bone marrow cells of a 75-year-old woman showed three different karyotypes, i.e., 46,XX,46,XX,t(1;3)(p36;q21) and 46,XX,t(1;3)(p36;q21),t(14;17)(q32;q21). She received no antileukemic therapy, and 1 year later, all her bone marrow cells were t(1;3)(p36;q21),t(14;17)(q32;q21). In association with the onset and first 11 months of ANLL, the platelet count increased 10-fold to a peak of 750 x 10(9)/L, providing further evidence that the t(1;3)(p36;q21) translocation causes stimulation of thrombopoiesis. Six months after transformation, her red cells showed reduced expression of A and Leb antigens. Serum alpha-n-3-acetylgalactosaminyl transferase (blood group A transferase) and red cell adenylate kinase were both reduced. The genes for both these substances are at 9q34, which suggests an abnormality here, although cytogenetically chromosome 9 appeared normal. This is the first case with t(1;3)(p36;q21) to show concurrent loss of red cell antigens and the first report detailing the course of untreated ANLL with t(1;3)(p36;q21).
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PMID:Acute leukemia with t(1;3)(p36;q21), evolution to t(1;3)(p36;q21), t(14;17)(q32;q21), and loss of red cell A and Le(b) antigens. 145 54

A total of 56 patients were diagnosed as primary myelodysplastic syndrome (MDS) at Chang Gung Memorial Hospital, Kaohsiung from April 1986 to December 1991. The median age was 65 years with an equal sex ratio. All patients presented with anemia and 52% with pancytopenia. The overall median survival for the entire group was 7 months, in which the chronic myelomonocytic leukemia (CMMoL) was 7 months, and 4 months for each of the refractory anemia with excess of blasts (RAEB) or the refractory anemia with excess of blasts in transformation (RAEB-T), however, the median survival had not been reached at 27 months for refractory anemia (RA) and at 33 months for refractory anemia with ring sideroblasts (RARS). Low-does arabinosyl cytosine (Ara-C) was administered in 9 patients with RAEB and RAEB-T, but no survival benefit was noted. Infection, especially pneumonia, was the most common cause of death. In 61 febrile episodes with clinically suspected sepsis, 10 (17%) were documented to associate with bacteremia. Twelve patients (7 RAEB, 4 RAEB-T, and 1 CMMoL) evolved to acute myelogenous leukemia (AML), the median interval from diagnosis to evolution was 4.8 months. This series indicates that only two groups of FAB subtypes could be clearly separated in terms of morphological findings and clinical outcome; RA and RARS constitute a good prognostic group, whereas RAEB, CMMoL, and RAEB-T constitute a poor prognostic group.
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PMID:Primary myelodysplastic syndrome: an analysis of 56 patients. 146 34


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