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

Although the FAB classification of the myelodysplastic syndromes (MDS) allows to classify most patients, a few clinical patterns do not fit the FAB categories, including borderline forms with manifestations usually seen in other diseases and forms with atypical or unusual clinical or laboratory features that do not immediately suggest a MDS. Borderline forms are characterized by the presence of any of the following: high or very high platelet counts, myelofibrosis, bone marrow hypoplasia, eosinophilia, or systemic diseases such as relapsing polychondritis. Unusual or atypical forms include manifestations such as hemolysis, high reticulocyte counts, erythroblastopenia, or an abnormality of a single cell line such as isolated thrombocytopenia, isolated neutropenia, or isolated macrocytosis. The definitive diagnosis of these forms of MDS can require a number of investigations such as cytogenetic studies, bone marrow biopsy, and/or radionuclide evaluation, and may not be possible until the patient has been followed for some time.
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PMID:[Myelodysplastic syndromes: unusual and mild forms]. 940 83

Erythrocyte deformability was determined in more than 500 clinical samples, and was found to be elevated in conditions in which fetal-like red cells are produced: aplastic anemia (3/3 cases), myelodysplastic syndromes, polycythemias, sickle cell anemia during treatment with hydroxyurea, paroxysmal nocturnal hemoglobinuria, and recovery from B12 deficiency. Elevated deformability was observed in neonatal erythrocytes, and during recovery from transient erythroblastopenia of childhood, when fetal-like red cells are known to be produced. Increased deformability appears to be a feature of fetal and fetal-like red cells. Forty-eight cases of enzymatically verified glucose-6-phosphate (G-6-PD) deficiency were also examined. Thirty out of 32 G-6-PD(A-) individuals, including both heterozygotes and hemizygotes, exhibited increased deformability during the steady state. In contrast, G-6-PD(Med) hemizygotes had normal deformability. Increased deformability was also found in G-6-PD(Huron) (n=3), G-6-PD(Wayne) (n=4), triose phosphate isomerase deficiency (n=2), and pyruvate kinase deficiency (n=2). An elevated osmoscan was found in more than 90% of female G-6-PD heterozygotes, affording a simple screening test for heterozygotes. Deformability remained high during hemolytic episodes, when older enzyme deficient cells are removed from the circulation. In four cases of G-6-PD deficiency with normal deformability, evidence for co-existing hereditary spherocytosis was found. The combination of conditions with opposing effects on deformability resulted in nearly normal deformability. Because increased red cell deformability is a feature of fetal erythrocytes, these results suggest that the red cells in many cases of glycolytic enzyme deficiency are fetal-like.
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PMID:Increased erythrocyte deformability in fetal erythropoiesis and in erythrocytes deficient in glucose-6-phosphate dehydrogenase and other glycolytic enzymes. 989 Jun 17

Myelodysplastic syndrome with erythroid hypoplasia or erythroblastopenia has not yet been clearly defined, and in most patients it is mistaken for acquired pure red cell aplasia. Including one additional patient reported in this article, a literature review revealed only 50 cases over the last 20 years. These patients were predominantly elderly males, all required regular packed red cell transfusions, and they had a poor prognosis, mainly because of acute transformation. The mechanisms of erythroid aplasia remain unclear. However, recent data suggest the association of an intrinsic stem cell defect with immunological implication.
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PMID:[Myelodysplastic syndrome with erythroblastopenia]. 2080 59