Risultati delle nostre osservazioni sulla Drepanocitosi e Thalassodrepanocitosi

Abstract

SUMMARYThe Authors studied 60 individuals bearing a positive test of sickle shaped cells (40 healthy bearers and 20 sick people).Drepanocytosis besides causing a Splenomegalic haemolitic disease in an homozygous state can also cause it in pure heterozygous individuals of high expressivity. Thalassodrepanocytosis due to a more frequent occurrence of thalassemia is found with a greater occurrence than sickle cell anaemia in Sicily.A patient showed a Salmonella osteomyelitis and two more a syndrome of acute abdomen due to a splenic occlusion.The study of myelograms showed in all the patients (drepanocytosis, thalassodrepanocytosis) a clear erythroblast hyperplasia.A cytometric study of the different aspects of the erythron was carried out.The study of Hb composition made through paper electrophoresis and the amount of alkali resistent Hb showed in the patients bearing sickle cell anaemia the presence of a unique band with the speed of Hb S and the coexistence of different amounts of Hb Ar (normal values to 33,6%observed in a pure etherozygous). Quantities of Hb Ar above the norm where always observed in thalassodrepanocytosis, but the electrophoretic finding, due to the coexistence of Hb A, ran from the shaded unique band to two distinct bands.