B. Dr. Marie Stuart and Dr. B.N. Setty spoke on Comparison of Hemoglobin SS and SC Adhesive Responsiveness. In sickle cell disease, loss of erythrocyte membrane phospholipid asymmetry occurs with the exposure of phosphatidylserine, which provides a docking site for coagulation proteins. F cells correlated inversely with both microvesicles and PS positivity in SS disease. Multiple regression analyses using various hematologic parameters as independent variables, and either microvesicles or PS positivity as the dependent variable, showed a strong relationship only with F cells. These levels of Hb F are generally present until about 1 year of age.
Similar findings occur in patients with SC disease but are quantitatively much less.
A. Dr. Steinberg will ask the VA to appoint his successor prior to the next meeting. This has been completed and Dr. Joseph De Simone will be assuming the position. B. Dr. Marie Stuart, Dr. Martin Steinberg, and Dr. Michael Terrin will consider the issue of a clinical study of hydroxyurea in hemoglobin SC disease in more detail. Dr. Stuart will return with a recommendation for the committee to consider. The issue was discussed in depth along with the discussion of the above presentation.