Hemoglobin K # { 246 } Inin a Black : Pre . . and Post - Splenectomy Red Cell Survival ( DF ” 2 P and 5 iCr ) and the Pathogenesis of Hemoglobin Instability

A 17-yr-old black male with hemolysis cells presplenectomy was 5.6%/day. and pigmenturia but no anemia was found whereas after surgery it was normal to Lave hemoglobin K#{246}In (a2 $ val-.met (1 .9%/day). accounting for the disparity EFG5I). Splenectomy was done because of between the survival methods. Study of the complicating thrombocytopenia. Thromboisolated cyanferri derivative of hemoglobin kinetic studies with Cr tagged platelets K#{246}lnby ultracentrifugation at various salt suggested hypersplenism. and after surgery concentrations and various pH’s indicated the platelet count returned to normal. The an increased tendency to dimer formation red cell t 4 51 Cr was more than doubled, under conditions where normal hemobut the red cell life span (DF32P) was globin is a tetramer. This results from the more modestly improved (30.6 47.2 site and type of amino acid substitution and days). The ‘elution” of Cr from the red accounts in part for its instability.