The kidney in thrombotic thrombocytopenic purpura.
The kidney is commonly affected in thrombotic thrombocytopenic purpura (TTP), a multi-system disorder with microvascular thrombosis of the capillaries and arterioles. Nevertheless, due to difference in its diagnostic criteria, the frequency and severity of renal dysfunction in TTP remains controversial. Recent studies indicate that the severe deficiency of a von Willebrand factor (VWF) cleaving protease, ADAMTS13, is the main cause of platelet thrombosis in TTP; it is now possible to define TTP at the molecular level. Among patients with acquired TTP due to inhibitory antibodies of ADAMTS13, renal dysfunction is usually mild; oliguria, fluid overload, hypertension, and need of dialysis support are infrequent. When any of these complications occur, one must re-examine the diagnosis of TTP and consider the possibility that the patient has another cause for these complications. In a patient with hereditary TTP, acute renal failure may ensue. However, the renal dysfunction is mostly reversible if the patients are promptly treated with plasma to replenish ADAMTS13. Patients with TTP, particularly of the hereditary type, may develop chronic renal failure. This complication may be a consequence of repeated insults by overt or subclinical microvascular thrombosis to the kidney, or it may have a separate cause. Therapy of hereditary TTP should aim not only to prevent acute exacerbations but also to minimize the risk of irreversible renal injury.
Tsai
H-M
HM
Montefiore Medical Center, Division of Hematology, Bronx, New York, NY 10467, USA. htsai@montefiore.org
D051722:ADAM Proteins / Q000172:deficiency* / Q000502:physiology; D000071120:ADAMTS13 Protein; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology* / Q000628:therapy; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012307:Risk Factors
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human