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Familial Thrombotic Microangiopathy

RICHARD H. MERRILL, CHARLES L. KNUPP, J. CHARLES JENNETTE
DOI: http://dx.doi.org/ 749-759 First published online: 1 November 1985

Abstract

SUMMARY A family in which there were two certain and three possible cases of thrombotic microangio-pathy in two generations is presented. All afflicted members studied presented with acute renal failure, and accelerated hypertension. No abnormal platelet-aggregating activity could be identified in the plasma of asymptomatic family members or in the surviving patient in remission. Although an increase in the Factor VIII: von Willebrand's factor level was found in the sole surviving patient, the multimer pattern was normal. Platelet-associated IgG level was also normal in this individual. Thus, individuals predisposed to this familial form of thrombotic microangiopathy do not have a demonstrable marker which can be implicated in the development of the disorder. Long-term study of this kindred is required to determine the factors which are important in the pathogenesis of thrombotic microangiopathy in the affected individuals.