Abstract
We studied 12 Hb C carriers: 4 homozygotic Hb CC and 8 heterozygotic. We observed the presence of free crystals in the peripheral blood of the homozygotes but in none of the heterozygotes. However, after incubation with 3% NaCl we were able to detect crystals in the heterozygotes (Hb AC and Hb SC), and in the homozygotes (Hb CC). In patient 04 (P04) less crystals formation occurred due to inhibition of the process by the presence of elevated levels of Hb F (12.2%). All the homozygotic patients had a splenomegaly of 3 to 6 fingerbreadths.We believe that the spleen wears off with time, thus allowing the passage of crystals to the peripheral blood. This finding might be associated with splenic insufficiency without a reduction of its dimensions. Finally, the finding of crystals in the peripheral blood permitted the diagnosis of Hb C obviating the need for electrophoresis.
Subject
Infectious Diseases,General Medicine
Reference17 articles.
1. The solubility of sickle cell and non sickle hemoglobin in concentrate phosphate buffer;ADACHI K.;J. biol. Chem.,1979
2. Hemoglobinas anormais em São Paulo. Métodos de estudo. Incidência;ARAÚJO J.T. de;J. bras. Med.,1965
3. Hemoglobinopatias: aspectos moleculares, genéticos e clínicos;ARAÚJO J.T. de;Rev. Hosp. Clín. Fac. Med. S. Paulo,1964
4. Estimation of small percentages of foetal haemoglobin;BETKE K.;Nature,1959
5. Pathogenesis of hemolytic anemia in homozygous hemoglobin C disease;CHARACHE S.;J. clin. Invest.,1967
Cited by
2 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献