Burkitt's lymphoma cell leukemia.
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Biomedical subjects
Publications and source records attributed to S Ozsoylu.
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The haematological and clinical data in 97 sickle-cell anaemia cases and haematological findings of their parents are reported. In spite of the low Hb values of the patients, they tolerated their anaemia and very rarely required blood transfusions. The Hb F levels of the patients were in general higher than African origin SS anaemia patients but lower than the Shiite Saudi Arabians. However, in most of the cases the concentration of Hb F did not seem to influence the Hb concentration of the patients. Serum iron was found unexpectedly decreased in 22% of the patients. Osmotic fragility was found decreased in 100% of the patients and in 83.5% of the parents. The prevalence of G-6-PD deficiency was 21.2% in male patients and 15.6% in the parents.
Mean platelet survival was significantly decreased (P less than 0.01) in three children with chronic and five children with acute ITP in remission. Sera from these patients sensitized normal platelets, as demonstrated by increased phagocytosis by autologous granulocytes. These results may suggest that the remission in some patients with ITP is obtained by compensatory platelet production.
The proband of each of three families of Northern European or Italian extraction had an unusual form of heterozygous beta-thalassaemia, confirmed by haematological, genetic and peripheral blood globin synthesis studies. The unusual severity of this disorder was indicated by chronic haemolysis leading to splenectomy and cholecystectomy, by numerous nucleated red cells and reticulocytes in the peripheral blood, and by leg ulcers in one family. The diversity of clinical expression in many family members with heterozygous beta-thalassaemia was striking. Bone marrow examination in the probands showed numerous large inclusion bodies of the type usually found only in thalassaemia major. In addition, there was unbalanced globin synthesis in the bone marrow, in contrast to the more balanced synthesis found in asymptomatic beta-thalassaemia trait. The amount of newly synthesized alpha-chain found in the free alpha-chain pool was markedly elevated. The unbalanced globin synthesis and alpha-chain inclusions in the bone marrow cells suggest that the severity of the disorder in these patients may be due to the inability of their red cell precursors to fully compensate for the thalassaemic defect or to remove excess alpha-chains. The diversity of clinical expression suggests the influence of undefined acquired or genetic factors on the expression of beta-thalassaemia in these families.
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Blood specimens were obtained from 281 inhabitants of an Eti-Turk village with a population of about 500. Starch gel (pH 8.6) and agar gel (pH 6.45) electrophoresis were performed in 279 of the specimens. Hb S was present in 105 partially interrelated persons (37.36%), three of whom had sickle-cell anaemia. Hb E was detected in 5 persons (1.79%), one of whom was a double heterozygote for Hb S and Hb E. One Hb S+alpha-thalassaemia and 7 Hb S with elevated Hb A'2 combinations were found. The beta-thalassaemia gene prevalence was 0.0377. Hb A2 was found in 4 persons (1.42%), and Hb F was slightly increased in 37 (22.3%) persons with a normal haemoglobin picture. Erythrocyte G-6-PD deficiency was 10% among males.
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