Priapism, a complication of male patients with sickle hemoglobinopathies, has been managed by a variety of surgical and nonsurgical forms of therapy that often are unsuccessful. The application of automated erythrocytopheresis (red blood cell exchange) by continuous-flow and semicontinuous-flow procedures appears to offer distinct advantages in the treatment of complications resulting from sickle hemoglobinopathies. The successful application of erythrocytopheresis for the relief of priapism in a patient with hemoglobin SC disease is presented and probably represents the first case reported on the use of automated red blood cell exchange procedures in the treatment of this condition. Data and results of automated erythrocytopheresis in 4 additional patients are presented. The advantages and disadvantages of erythrocytopheresis in the treatment of priapism (and other complications of sickle hemoglobinopathies) are discussed and the method is compared to other modes of therapy.
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.
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