Congenital Deficiency of a Factor in Normal Plasma That Reverses Microangiopathic Hemolysis and Thrombocytopenia

Abstract

THE patient described below has been under observation for 11 years. Evidence to be presented suggests that she has the congenital deficiency of a factor in normal plasma that appears under some circumstances to be important in platelet and red-cell survival.Case ReportThe patient, now 29 years old, was noted at birth to have a rudimentary right radius and ulna, and a lobster-claw deformity of the right hand.From the age of six months to 12 years she had six to 10 episodes yearly characterized by the acute onset of high fever, generalized petechial rash, usually a clinically determinable . . .