Renal papillary necrosis in sickle-cell haemoglobinopathy.

Abstract

Abel and Brown (1948) first drew attention to profuse haematuria in sickle-cell anaemia. Subsequent reports have confirmed that this association is more than fortuitous (Harrison and Harrison, 1952;Chapman et al., 1955 ; Sharpe et al., 1959). Haematuria is also known to occur in sickle-cell trait, sickle-cell haemoglobin C disease, sickle-cell thalassaemia, and a variety of combinations of haemoglobin S with other abnormal haemoglobins. The present report of four cases of haematuria in sickle-cell haemoglobinopathies is prompted for two reasons. Firstly, detection of blood in the urine of young adults in an environment endemic for schistosomiasis need not always indicate the presence of the infection. Of more practical importance is the surgical diagnostic problem sometimes posed by gross haematuria simulating renal neoplasm. Awareness of the possibility that this symptom may exist in sickle-cell haemoglobinopathy makes careful appraisal necessary, to avoid the inadvertent sacrifice of an apparently normal-functioning kidney. (Fig. 1). Her haematuria cleared gradually within a fortnight and she has since remained well and active.Case 2 A tailor aged 47 was subject to recurrent episodes of painless haematuria for two months. Examination revealed severe anaemia (Hb 3.0 g./100 ml.). His urine contained a trace of albumin and its S.G. was 1006; red blood cells were detected at microscopy, but there were no casts. Blood group was O-positive, sickling was present, and his genotype AS. His blood urea was 16 mg./100 ml. Cystoscopy showed blood spurting from the left ureteric orifice. Left ureteric catheterization was followed by a retrograde pyelogram, after which a nephrectomy was performed. Naked-eye appearance of the kidney showed marked congestion of the vessels close to the papillae, with softening of the central and apical portions of the pyramid; there were blood clots in the renal pelvis. Microscopy did not reveal any abnormalities in either glomerular or tubular morphology. He remained well for five years, but has lately developed proteinuria and hypertension. The latter event has been complicated by congestive heart failure, but this has responded well to digoxin, thiazides, and reserpine. At the time of writing he remained practically symptom-free.