beta+-Thalassaemia, Haemoglobin S and Hereditary elliptocytosis in a Zairian Family. Ischaemic costal necroses in a child with sickle cell beta+-Thalassaemia.
In a family of Low-Zaïre, a child with sickle cell beta+-thalassaemia disease, aged 7, presented 33.6% haemoglobin A. The clinical evolution was benign, except for the occurrence of an extensive bilateral ischemic necrosis of the ribs. This is the third case of sickle cell thalassaemia with the haemoglobin phenotype SFA reported in Zaïrians, whereas the SF phenotype has repeatedly been found: beta+-thalassaemia seems therefore much rarer in Zaïrians than betadegrees-thalassaemia. Besides the thalassaemia trait and the sickle cell trait, hereditary elliptocytosis was also segregating in this family; double heterozygotes for Hb S and elliptocytosis or for beta-thalassaemia and elliptocytosis did not show any definite sign of genetic or clinical interaction between both traits.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000235:genetics*; D002648:Child; D015023:Democratic Republic of the Congo; D004612:Elliptocytosis, Hereditary / Q000097:blood / Q000235:genetics*; D005260:Female; D006455:Hemoglobins, Abnormal / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D010375:Pedigree; D011859:Radiography; D012272:Ribs / Q000000981:diagnostic imaging; D012805:Sickle Cell Trait / Q000097:blood / Q000235:genetics*; D013789:Thalassemia / Q000097:blood / Q000235:genetics*
D006455:Hemoglobins, Abnormal