Purpura fulminans in severe congenital protein C deficiency: Monitoring of treatment with protein C concentrate

Treatment of inherited protein C deficiency by replacement therapy with the French purified plasma‐derived protein C concentrate (PROTEXEL®)

“…The treatment of an acute thrombotic event in case of severe PC deficiency is replacement therapy [3,[11][12][13][14][15][16][17]. Correction of PC deficiency achieved using PC concentrate leads to complete resolution of the DIC [11,[13][14][15][16]. When it is initiated at an early stage after the onset of the thrombotic lesions, it leads to a complete recovery without sequelae [15].…”

Mice with a severe deficiency in protein C display prothrombotic and proinflammatory phenotypes and compromised maternal reproductive capabilities

“…The importance of the PC pathway in regulating hemostasis is clear in clinical settings wherein patients identified as having congenital or acquired deficiencies in this pathway present with thrombotic complications, e.g., superficial and deep vein thrombosis (7), pulmonary embolism (8), purpura fulminans (9), and, occasionally, arterial thrombosis with resulting stroke (10) and/or pulmonary arterial hypertension (11). More recently, a direct or indirect role for aPC in inflammation has been increasingly recognized, and it is known that this enzyme plays an important role in protecting the host against bacterial infection (12)(13)(14).…”

De larges plaques ecchymotiques révélant un déficit homozygote en protéine C

“…Une fois le diagnostic de déficit en protéine C confirmé, le traitement repose sur des perfusions de plasma frais congelé [2]. Une autre alternative, plus récente, est les concentrés de protéine C, à la dose initiale de 100 UI/kg suivie de 50 UI/kg par six à 12 heures [4,5].…”