Decreased erythrocyte survival in hemoglobin H disease as a result of the abnormal properties of hemoglobin H: the benefit of splenectomy.

With the Technical Assistance of George Cummings, Marze L. Duersl1 Donald R. Maim, Katherine Swisher and Patricia Vanbeilinghen S INCE the first description of hemoglobin H,1 many reports have appeared indicating a wide racial and geographical distribution of this hemoglobinopathy. We reported a shortened erythrocyte survival in a case, which was attributed to the property of hemoglobin H to denature and precipitate.2 The spleen was thought to be the major site of destruction, and splenectomy was considered. Reports of improvement of patients with hemoglobin H following splenectomy:i4 strengthened this hypothesis. We will present data indicating considerable improvement of two patients with hemoglobin H disease following splenectomy and suggesting that the abnormal structure of hemoglobin H is the cause of the reduced erythrocyte survival and that its instability is enhanced by environmental factors, particularly by certain chemicals and drugs.