Structure - Function Relations of Human Hemoglobin
“Most of the abnormal hemoglobins with increased oxygen affinity manifest themselves by causing polycythemia in the carrier. The increased oxygen affinity reduces tissue oxygen delivery, causing an increase in erythropoietin secretion and in red cell mass. The possibility of an abnormal hemoglobin with high oxygen affinity should be considered in those atypical patients with polycythemia in which the white blood cell and platelet counts are not elevated and splenomegaly is absent. The importance of establishing the correct diagnosis is mainly to protect the patient from the chemotherapeutic treatment of polycythemia. Family members should be advised that their children may be affected. The life expectancy of affected individuals is essentially normal, and most patients are symptom free. However, if such patients become symptomatic and their hematocrit rises towards 60%, then phlebotomy may be necessary to reduce blood viscosity.”
