Renal papillary necrosis in hemoglobin SC disease.

Abstract

Sickle cell disease was first described by Herrick (9) in 1910. In 1949, Pauling (13) documented the anemia's molecular basis with the development of hemoglobin electrophoresis. Other hemoglobins have been discovered since then. Itano (10) in 1951 described hemoglobin C, which produces no symptoms when combined with the normal hemoglobin A but when combined with sickle cell hemoglobin S may produce hemoglobin SC disease, an established clinical and radiographic entity. Renal papillary necrosis was originally ascribed to infection and was thought to be associated with rapid uremia and death. It is now considered to be a complication in many disorders, including diabetes mellitus, obstructive uropathy, phenacetin toxicity, renal vein thrombosis, alcoholism, pyelonephritis, and sickle cell trait or disease (17). While hematuria is common in hemoglobin SC disease, several reports state that there are no radiographic findings in the urinary tract (2, 14). There is only one radiological report demonstrating renal medullary necrosis in this condition (17). Other radiographic findings in hemoglobin SC disease have been described previously (1, 2, 6, 11, 14–16). It is our purpose to present a case of both renal papillary necrosis and osseous changes in a patient with hemoglobin SC disease. Case Report I.C., a 29-year-old Negro female, presented with a three-week history of hematuria. She passed clots in her urine one week prior to admission, at which time right paralumbar and right upper quadrant pain developed. There was no past history of hematuria or calculus. The patient complained of moderate chronic exertional dyspnea. No history of bone or joint pains was elicited, but there has been intermittent swelling of the left ankle. The patient recalled receiving blood transfusions for anemia when she contracted meningitis at thirteen years of age. Her mother has diabetes mellitus, and one brother is known to have anemia, as do two of her children. Physical examination revealed an afebrile female in no acute distress. Blood pressure was 112/70, and the pulse rate was 98 per minute. She had a Grade 2 systolic murmur. The spleen was palpable. The liver was not enlarged. Hemoglobin was 7.2 g per 100 cc. The white blood cell count was 7,000, with a normal differential distribution. The erythrocytes were hypochromic, and target cells and sickling were observed on the peripheral smear. The serum iron was 40 µg per 100 cc. Blood glucose and blood urea nitrogen were normal. Nonhemolytic streptococci were cultured from the urine on one occasion and were felt to be a contaminant. Repeat urine cultures were sterile. Hemoglobin electrophoresis was performed by two independent laboratories and revealed the presence of hemoglobin SC. The patient's mother had hemoglobin CA. Two sons available for testing had types SC and CA respectively.