Intraerythrocytic Hemoglobin Crystals in Sickle Cell-Hemoglobin C Disease

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Red cell life span in sickle cell-hemoglobin C disease with a note about sickle cell-hemoglobin O ARAB.

Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2-121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9...

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Hemoglobin S gelation and sickle cell disease.

By William A. Eaton and James Hofrichter T HE FUNDAMENTAL cause ofsickle cell disease is the decreased deformability of the sickled red cell produced by gelation of hemoglobin S. Partial inhibition of gelation should therefore reduce clinical severity, while complete inhibition should result in a “cure.” These basic ideas have stimulated an enormous effort to understand the gelation process in ...

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ژورنال

عنوان ژورنال: Blood

سال: 1965

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v25.2.218.218