Article
Protein C deficiency.
Haemophilia : the official journal of the World Federation of Hemophilia - 1 Nov 2008
Goldenberg N A, Manco-Johnson M J
Abstract excerpt
Severe protein C deficiency (i.e. protein C activity <1 IU dL(-1)) is a rare autosomal recessive disorder that usually presents in the neonatal period with purpura fulminans (PF) and severe disseminated intravascular coagulation (DIC), often with concomitant venous thromboembolism (VTE). Recurrent thrombotic episodes (PF, DIC, or VTE) are common. Homozygotes and compound heterozygotes often possess a similar...
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