Protein C survival during replacement therapy in homozygous protein C deficiency

Homozygous protein C (PC) deficiency is a rare genetic defect that usually results in fatal thrombotic complications (purpura fulminans and DIC), but it can be successfully managed with oral anticoagulants or PC replacement. The successful use of PC replacement for two individuals is described. The activity and antigen levels of PC in fresh frozen plasma (FFP) and prothrombin complex concentrate (PCC) are also reported. The concentration of PC in FFP is 87 ± 15 units/dl. PC is present in all PCC analyzed; however, a ten‐fold difference between the various brands and/or lots is noted. The PC activity and antigen correlates well with no significant levels of APC. Upon infusion of FFP into two homozygous PC‐deficient children, the PC levels obtained were ≦30 units/dl post‐ infusion and undetectable after 12‐18 hr. With infusions of PCC, plasma levels of PC obtained were 100‐145 units/dl and