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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of the present work was to perform a prospective analysis of the significance of macrocytic red cells through the study of all patients with MCV higher than 105 fl (those treated with cytotoxic or immunosuppressing drugs were excluded). Conventional clinical, haematologic and biochemical studies were carried out on every patient, along with B12 and folate levels, bone marrow examination and bone marrow karyotype and, whenever B12 deficiency was present, complete Schilling's test. Special attention was paid to the aetiological inquiry and post-therapeutical course. A series of 109 patients was collected. Decreased serum B12 rates with abnormal Schilling's test and response to parenteral therapy were present in 26 cases (24%). Of them, 22 fulfilled the diagnostic criteria for Biermer's anaemia, while in the remaining 4 there was impaired intestinal absorption. Serum or red-cell folate deficiency was found in 34 other cases (31%). Alcoholism was present in 20 of them, abnormal diet in 10, malabsorption syndrome in 2, and excessive demands in 2 others. Hence, vitamin deficiency underlay macrocytosis in 60/109 cases (55%). In the remaining 49 cases (45%) macrocytosis was not accompanying folate or B12 deficiency. Of these, severe liver disease was found in 16 patients (alcoholic in 15 and post-hepatitis in 1 case), with increased serum B12 in 10 cases and increased serum or erythrocytic folate in 3 others. Nineteen patients within this group had primary myelodysplastic syndromes (RA, 8; SRA, 4; RAEB, 7), and the remaining 14 cases had several haematological (AIHA, 4; CLL, 1, T-cell lymphoma 1, M-6, 1, and myelofibrosis with myeloid metaplasia, 2) or non-haematological diseases (heart insufficiency, 2; COPD,3).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Hematologic significance of erythrocytic macrocytosis: prospective analysis of 109 successively studied cases]. 271 Dec 82

Urinary methylmalonic acid (MMA) excretion in megaloblastic anemia due to vitamin B12 (B12) deficiency was studied using a colorimetric method. Average MMA excretion in 20 patients with untreated B12 deficiency was 164 mg/day, whereas it increased to 518 mg/day following oral administration of 10 g L-valine. Urinary MMA correlated significantly with platelet number, erythroblast percentage and deoxyuridine suppression test, while no correlation was found with hemoglobin, leukocyte number, reticulocyte, serum LDH, serum B12 and folate concentration. Patients with neurological disturbances excreted significantly larger amounts of MMA than those without neurological disorders. The results also indicated that MMA could be a useful adjunct for differentiation of megaloblastic anemia from myelodysplastic syndromes showing marked megaloblastic changes.
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PMID:[Urinary methylmalonic acid excretion and clinical features in megaloblastic anemia due to vitamin B12 deficiency]. 279 94

The association between leukemic transformation and various features recorded at presentation in patients with refractory anemia with excess of blasts and with or without ringed sideroblasts was analyzed in 255 patients using the proportional hazard model. Features associated with higher transformation rates were: higher values of blasts in peripheral blood or bone marrow; serum haptoglobin; vitamin B12; megakaryocytes in bone marrow; morphological abnormalities in granulo- or megakaryocyte series; male sex; circulating megakaryocytes in peripheral blood; older age; and lower ringed sideroblast proportion. Multivariate analysis was also performed using the following predictor variables: presence or absence of refractory anemia with excess of blasts; sex; abnormal granules in granulocytes; age; and mononuclear large megakaryocytes. Patients were divided arbitrarily into low (hazard ratio, less than 0.45), intermediate (hazard ratio, 0.45-1.85) and high (hazard ratio, greater than 1.85) risk groups. The cumulative leukemia-free rates in the low and intermediate risk groups showed long plateau phases at 95 and 71%, respectively, while in the high risk group, the rate was 10% at 5 years. For clinical purposes, the low risk group should be considered to have nonpreleukemia and the high risk group to have preleukemia.
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PMID:Factors influencing leukemic transformation in refractory anemias with excess of blasts, with ringed sideroblasts, and without ringed sideroblasts. 345 28

Chronic myelomonocytic leukemia (CMML) is a rare leukemia, which is now included in myelodysplastic syndromes. In a small number of patients with CMML, problems in the diagnosis have been reported, especially when atypical morphological features in both monocytic and granulocytic cells due to dysmyelopoiesis are prominent, or when cytochemical characteristics are lost in the leukemic cells. The case history of a sixty-seven year-old male patient with CMML is described. The diagnosis of CMML in the patient was supported by the following evidence: chronic course of his disease; increased monocyte-like cells without other cause; normocytic anemia; immature granulocytic cells with hypogranular feature and giant platelets were observed in the peripheral blood. The bone marrow showed myeloid hyperplasia. Serum muramidase and vitamin B12 levels were increased, while neutrophil alkaline phosphatase score was low in the peripheral blood. Ph' chromosome was negative. The monocyte-like cells completely lacked nonspecific esterase. However the cells were confirmed as monocytic cells by flow cytometry using monoclonal antibodies to monocytes (OKM5).
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PMID:Report of a case with chronic myelomonocytic leukemia: demonstration of leukemic monocytes lacking nonspecific esterase by flow cytometry using monoclonal antibodies. 350 52

Two cases of myelodysplastic syndrome in the dog are described. Both cases were characterized by hepatomegaly, cytopenias, macrocytosis, cellular bone marrow, and dysplastic changes in all hematopoietic cell lines. Serum vitamin B12 and folic acid concentrations were within reference ranges in one dog, but vitamin B12 was slightly decreased in the other. Vitamin B12 therapy did not result in amelioration of the hematologic disorder. The duration of illness was approximately 5 months. Necropsy of one dog revealed an acute undifferentiated leukemia.
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PMID:Myelodysplastic syndrome in two dogs. 406 60

Three patients with idiopathic pancytopenia and hypercellular bone marrow who developed carcinoma of the lung within two years of diagnosis are reported. All three patients had macrocytic anemia associated with a megaloblastic marrow in the presence of normal serum vitamin B12 and folic acid levels. Neutropenia with monocytosis, elevated serum muramidase and LAP scores, and increased fetal hemoglobin levels were also found. In all cases Ham's tests were negative with a normal bone marrow karyotype. In all three patients, pancytopenia due to myelodysplasia, a probable preleukemic state, was diagnosed initially prior to the appearance of carcinoma of the lung. One of the patients showed improved leukocyte and platelet counts during chemotherapy, while the other two died before chemotherapy could be administered. In the light of the above findings we suggest that carcinoma of the lung may be the cause of a paraneoplastic syndrome with pancytopenia, particularly in patients with a hypercellular marrow with a normal karyotype.
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PMID:Pancytopenia with hypercellular bone marrow--a possible paraneoplastic syndrome in carcinoma of the lung: a report of three cases. 672 Jun 84

Six children who developed acute myelomonocytic leukemia presented with a preleukemic syndrome. The incidence of preleukemic presentation of AMML was 17% of children with acute nonlymphocytic leukemia, or 2.9% of all children with acute leukemia at this institution, incidences similar to those in adults. During the preleukemic phase, which lasted from three to 35 months, all children had anemia, all had infectious complications, and three of six had hemorrhagic tendencies. Three received steroids before the diagnosis of AMML, and all had some objective response. Two patients died before receiving therapy for AMML. Four children who received AMML therapy with combinations including cytosine arabinoside and an anthracycline achieved complete remission. Ultimately, all patients died. Clues to diagnosis of preleukemia include unexplained cytopenias, either absolute or functional, peripheral blasts, progressive megaloblastosis with an elevated B12 value, dyserythropoiesis, abnormalities of nuclear segmentation, nonrandom chromosomal alterations, and reduced marrow colony to cluster ratio in vitro. Until there is a highly effective therapy for ANLL, precisely when to treat the child with preleukemia remains uncertain. However, treatment should be started before infectious complications or hemorrhagic tendencies become life-threatening.
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PMID:Preleukemia in children. 720 82

The cause of macrocytosis (mean corpuscular volume (MCV) greater than or equal to 105 fl) was investigated over a period of 16 months in the 70 known cases of +/- 4000 patients seen by the Department of Internal Medicine. By means of simple laboratory investigations the cause of elevated MCV was found in more than 90% of the cases. We found vitamin B12 or folic acid deficiency in 27 patients, alcohol abuse in 18, chronic persistent hepatitis in 2, hematological (pre-) malignancy in 9, hemolysis in 4, hypothyroidism in 2, and a drug effect in one patient. In 6 cases the elevated MCV could not be explained. Macrocytosis, a frequent finding that is not related to the hemoglobin concentration, is an indicator of serious pathology. The MCV level can be used to differentiate between the diagnostic categories. Only 21 patients (30%) had megaloblastic erythropoiesis that was difficult to recognize in the peripheral blood. Among the findings at routine laboratory investigations an elevated MCV may be the only indicator of vitamin deficiency, preleukemia or alcoholism.
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PMID:The clinical significance of macrocytosis. 723 9

Deoxyuridine (dU) suppression test and granulocyte macrophage colony forming unit (CFU-GM) assay were performed, along with morphological analysis, vitamin B12 and folate assays, in 22 cases of macrocytic anaemia corresponding to various types of idiopathic dysmyelopoietic syndromes. The dU suppressive effect was found to be normal in 19 cases, and slightly subnormal in 3 cases. The parallel study of the in vitro bone marrow growth showed, except in cases of primary acquired sideroblastic anaemia, a reduced number of colonies associated with a relative increase of the cluster formation. Consequently, these two parallel functional studies can support a formal diagnosis of potentially preleukaemic myelodysplasia, whatever may be the morphological pattern and even in cases of marked macrocytic anaemias and secondary epiphenomenal folate or vitamin B12 deficiency.
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PMID:Deoxyuridine-suppression test and bone marrow culture for the diagnosis of macrocytic refractory anaemias. 746 11

Because of the widespread use of multiparameter hematology instruments, physicians are often presented with patients who have macrocytosis with no obvious cause. Depending on the demographics of an individual practice, folate and vitamin B12 deficiencies may be relatively rare causes of macrocytosis, compared with alcoholism, liver disease, drugs, or myelodysplasia. Initial evaluation should include a carefully taken history and physical examination along with a complete hematologic profile, reticulocyte count, and peripheral blood smear. This initial evaluation should allow systematic consideration of the possible causes. Serum B12 and red cell folate determinations and other studies may then be undertaken as appropriate.
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PMID:A systematic approach to macrocytosis. Sorting out the causes. 775 41


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