The management of hematuria associated with sickle hemoglobinopathies.

Hematuria secondary to sickle cell hemoglobinopathy was first reported by Abel and Brown in 1948. They noted sickled erythrocytes in a kidney removed for suspected tumor. Since then there have been numerous reports attributing hematuria to the abnormal hemoglobins that produce sickling of red cells. Although presently a well established entity the management of this fairly unique form of hematuria is far from standardized. The pathogenesis of the condition is fairly well understood and several modalities of treatment have been tried--many of which have a sound physiological basis in their use but a randomized prospective study in a large series of patients has yet to be performed. Herein is reviewed the current concepts of the etiology, diagnosis and management of this unusual form of hematuria.