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The kidneys in paroxysmal nocturnal hemoglobinuria
DA Clark, SA Butler, V Braren, RC Hartmann and DE Jenkins
Long-term study of 21 PNH patients revealed an unexpectedly high incidence
of functional and anatomic renal abnormalities. Most patients demonstrated
varying degrees of hematuria and proteinuria distinct from hemoglobinuria.
Evaluation of renal function revealed hyposthenuria, abnormal tubular
function, and declining creatinine clearance. Radiologically these patients
had enlarged kidneys, cortical infarcts, cortical thinning, and papillary
necrosis which were confirmed by autopsy studies. Hypertension developed in
eight patients. Urinary tract infection was uncommon. The renal findings
bear striking similarity to those of sickle cell anemia. Contrary to the
usual opinion, out studies clearly showed evidence of widespread renal
pathology in PNH most likely due to repeated microvascular thrombosis
similar to the venous thrombosis involving other organs in this disorder.
Volume 57,
Issue 1,
pp. 83-89,
01/01/1981
Copyright © 1981 by The American Society of Hematology
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