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

By way of introduction, the physiologic alterations of blood cells in old age are described. Besides the well known anemias in younger persons, protein deficiency may be an additional cause of anemia in the elderly. Acquired sideroblastic anemia of varying etiology is more often seen in the elderly than in younger people. In pernicious anemia the daner of gastric cancer has been overestimated. Aggressive treatment of acute leukemias is not indicated in patients over 60. The special form of smouldering leukemia is mentioned. The syndrome of anemia, thrombocytopenia and enzymatic dysfunction of granulocytes may, it is suggested, be a symptom of preleukemia. Anemia with accelerated sedimentation rate responsive to steriods is helpful in diagnosing polymyalgia rheumatica in the oligosymptomatic form.
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PMID:[Hematological problems in geriatrics]. 105 30

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

The DNA content of erythropoietic cells from 10 patients with refractory anaemia (RA) with megaloblastic changes, who subsequently developed acute non-lymphoblastic leukaemia (ANL), and from seven patients with megaloblastic marrow aspirates due to pernicious anaemia were compared by DNA image cytometry. The DNA distribution, the rate of aneuploid cells exceeding 5c (5cER), and the square deviation index of DNA values from the normal 2c-peak (2cDI) were recorded. Both variables were of diagnostic and prognostic importance for epithelial tumours, malignant lymphomas, and dysplastic lesions. A rate of 5cER greater than 0 was found in eight of 10 myelodysplastic, but in none of seven control cases. Hypodiploidy was equally pronounced in both groups of patients. The 5cE had the highest discriminative value of all variables calculated. The 2cDI was not significantly different in either group. In pernicious anaemia the 2cDI depended mainly on the percentage of S cells, reflecting the defect of DNA synthesis. In RA with megaloblastosis the 2cDI correlated with the percentage of G2 cells, reflecting G2 arrest. In the myelodysplastic group the 2cDI correlated positively with the length of time until ANL developed, indicating the prognostic relevance of 2cDI. Our findings show that in megaloblastic anaemia DNA image cytometry can distinguish myelodysplasia from pernicious anaemia and that it also provides prognostic information.
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PMID:Diagnostic and prognostic value of DNA image cytometry in myelodysplasia. 338 94

During a 5 year period, 28 adult patients with megaloblastic anaemia (MA) were treated in University Hospital. 71% of the patients were Indians. Symptoms of anaemia was the main presenting complaint in 18 (64%) of patients while in 2 patients peripheral neuropathy was the main problem. Pancytopenia was a common finding (present in 18 (64%) patients) while 6 (21%) patients had severe thrombocytopenia (less than 20 x 10(9)/L). The peripheral blood morphology provided important diagnostic clues i.e. macrocytes and/or hypersegmented neutrophils seen in most patients. Concurrent iron deficiency 'dampened' the megaloblastic picture. Though most patients suffered from MA of nutritional origin, 3 patients were diagnosed to have pernicious anaemia and 2 patients had myelodysplastic syndrome. Important practice points were detection of concurrent infection and hypokalemia which necessitated appropriate treatment. The controversy of blood transfusion in treatment of MA and the importance of reassessing patients after treatment were highlighted.
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PMID:Megaloblastic anaemia--a review from University Hospital, Kuala Lumpur. 340 25

The immunoglobulin levels and autoantibody profiles of 104 patients with primary myelodysplastic syndromes (MDS), classified according to the FAB criteria, were analysed. Eight patients, four with coexistent non-Hodgkin's lymphomas, three with chronic lymphocytic leukaemia and one with a lymphoplasmacytoma, were excluded from the final analysis of immunoglobulin levels. Serum protein electrophoresis and immunoelectrophoresis revealed the presence of monoclonal gammopathy in 12 patients (12.5%). Of the remaining 84 patients, a polyclonal rise in serum immunoglobulins was present in 27 (32%) while a further 16 (19%) had low immunoglobulin levels. The direct antiglobulin test was positive in eight out of 98 patients tested (8.1%), and organ and non-organ specific autoantibodies were present in 15 out of 67 patients tested (22.3%). Two patients had associated pernicious anaemia (PA), two hypothyroidism, and one PA with hypothyroidism. Three patients had sero-negative rheumatoid arthritis. These results demonstrate that there is a high incidence of immunological abnormalities in MDS.
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PMID:Immunological abnormalities in myelodysplastic syndromes. I. Serum immunoglobulins and autoantibodies. 370 59

The activity of the DNA excision repair enzyme uracil-DNA glycosylase was measured in peripheral blood mononuclear cells and in bone marrow aspiration samples obtained from patients with pernicious anemia (PA) or other types of megaloblastic anemia (one case of tapeworm anemia and three cases of myelodysplastic syndromes). In addition, the expression of uracil-DNA glycosylase was investigated in biopsies from the antrum and body of the stomach obtained from nine PA patients, from five patients having atrophic gastritis (AG) not associated with PA, and from six control patients having transient upper abdominal complaints without AG. Our results revealed that there was a considerable interindividual variation in gastric uracil-DNA glycosylase activity. No clear correlation between the enzyme level and the level of gastric atrophy was noted, although AG is generally regarded as a risk factor of gastric cancer. Furthermore, uracil-DNA glycosylase activities in peripheral blood mononuclear cells and in bone marrow cells in PA and in myelodysplastic syndromes were similar to the activities observed previously in non-hematological patients and healthy persons. Transient uracil incorporation into DNA may have a role in the cellular abnormalities associated with megaloblastic hematopoiesis. The present findings demonstrated that the enzymatic activity required for rapid removal of uracil from DNA is also expressed in the megaloblastic state.
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PMID:Hematopoietic and gastric uracil-DNA glycosylase activity in megaloblastic anemia and in atrophic gastritis with special reference to pernicious anemia. 380 19

Hyperthyroidism can be associated with various haematological disorders related to several mechanisms. These disorders might be related to the reduced life-span of whole blood components and/or to an autoimmune mechanism. Only one case of pancytopenia has yet been reported. The observation of 3 new personal cases (1 toxic adenoma and 2 Graves' disease) led us to review the pathogeny of haematological disorders found in hyperthyroidism. Only one patient had antineutrophil autoantibodies. Direct and indirect Coomb's test, and Dixon's test were negative. In all patients, bone marrow aspiration was unable to demonstrate pernicious anaemia or myelodysplastic syndrome. Two patients presented cytological signs of macrophage activation with eosinophilia. These cytological features were compatible with an immuno-allergy mechanism. All haematological disorders disappeared when patients became euthyroid. In all cases, the haematological abnormalities were quite mild and might have gone unnoticed. Thus, it can be suggested that the frequency of pancytopenia in hyperthyroidism is underestimated.
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PMID:[Pancytopenia resolved by the treatment of hyperthyroidism]. 763 Aug 70

The myelodysplastic syndromes (MDS) are neoplastic disorders of the hemopoietic system; multilineage involvement is also evidenced by specific cellular dysfunctions. The von Willebrand factor (vWF), synthesized and processed in the megakaryocytes (MK), is stored in the alpha granules of the platelets. The platelet vWF multimeric pattern was studied in 18 patients with MDS, and in 4 with pernicious anemia (PA), to investigate whether the processing of vWF is abnormal in the megakaryocytic dysplasia. An abnormal multimeric pattern was observed in 10/18 MDS and 4/4 PA patients. The abnormality of this specific protein is the discrete expression of the basic disorder, and is reversible when hemopoiesis is normalized. Although the data do not allow any conclusion, abnormal synthesis is the likely explantation of the abnormality.
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PMID:Abnormal platelet von Willebrand factor (vWF) as a marker of abnormal function in megakaryocytic dysplasia. 786 22

A total of 41,553 bone marrow biopsies were collected for the Bone Marrow Registry from January 1989 to June 1994 included 6,366 taken from 5,011 patients referred because of unexplained anemia. An increasing percentage of biopsies submitted for examination are designated anemic: this rose from 7.2% in 1989 to 18.9% within the first 6 months of 1994, reflecting an increased need to find the reasons for anemic conditions. Histologically, seven main groups have been found: (1) myelodysplastic syndrome (MDS) accounting for 32% of all anemia patients seen, (2) infectious anemia (23.5%), (3) iron deficiency anemia ((22.7%), (4) hemolytic anemias (7.2%), (5) aplastic anemia (6.8%), (6) megaloblastic/pernicious anemia (5.0%), and (7) anemia due to bleeding (3.0%). These seven groups of anemia can be diagnosed in core biopsies on the basis of their particular histopathology, so that films of bone marrow smears are not always needed.
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PMID:[The histological picture of anemia in bone marrow biopsies]. 788 14

An immunohistochemical and morphometric analysis was performed on bone marrow trephine biopsies in 40 patients with primary myelodysplastic syndromes (MDS) to evaluate the proliferative activity in erythropoiesis and the endoreduplicative capacity of megakaryocytes. Control groups included normal bone marrow and marrow from cases presenting with pernicious anaemia. Double-immunostaining was applied with a monoclonal antibody (PC10) directed against proliferating cell nuclear antigen (PCNA), followed by antibodies against glycophorin C (Ret40f) or platelet glycoprotein IIIa (Y2/51-CD61) for the identification of the erythroid and megakaryocytic cell lineage. Comparison with normal bone marrow showed a reduction of erythropoiesis accompanied by an increase in atypical (micro-) megakaryocytes. Erythroid precursors displayed significant enhancement of PCNA-immunostaining. Megakaryocytes showed no increase in the relative frequency of PC10-positive cells (PCNA-labelling index). In pernicious anaemia, predominance of macrocytic-megaloblastoid erythropoiesis was associated with a striking increase in PCNA-labelling. Cell kinetic studies in this disorder revealed an abnormal arrest, particularly in S-phase which generates an over-expression of PCNA. Similar conditions were believed to be present in MDS with secondary folate deficiency. This mechanism explains the relatively high rate of positively-reacting pro- and erythroblasts which is not invariably accompanied by an increase in cell proliferation. Determination of megakaryocyte size and PCNA-staining capacity resulted in a significant increase in PC10-positive cells among micromegakaryocytes. Our findings on this cell lineage are in keeping with the assumption of a block in endoreduplicative activity at higher ploidy levels, associated with an apparently not-deregulated endomitosis in small-sized megakaryocytes of lower ploidy stages.
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PMID:Myelodysplastic syndromes: immunohistochemical and morphometric evaluation of proliferative activity in erythropoiesis and endoreduplicative capacity of megakaryocytes. 810 4


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