1464: Antithrombin Iii Infusion in Pneumococcal Purpura Fulminans & Disseminated Intravascular Coagulation

Introduction: Purpura fulminans (PF) is a syndrome of microvascular thrombosis and hemorrhagic infarction of the skin associated with disseminated intravascular coagulation (DIC). Deficiency of Protein C or anti-thrombin III (AT-III) is associated with severity of PF in DIC but routine use of these agents to prevent or treat systemic thrombosis is controversial. We present a successful use of AT-III in PF due to pneumococcal septicemia. Description: 56 y-o man with past history of childhood splenectomy presented with acute onset of bluish discoloration of face, nose, fingers and toes. He had no fever, dyspnea or cough. In the ER, he developed severe hypotension and altered mentation. Purpuric lesions rapidly increased to involve face, neck, wrists and ankles. Due to cardiovascular collapse, he was intubated and resuscitated. Lactate was 2.5 mmol/l, WBC 21 K/cumm, platelets 42 K/cumm, fibrinogen was 59 mg/dl, PTT 75 seconds, D-dimer 55,200, INR 2.19. Liver enzymes were normal. Blood cultures grew Strep pneumoniae. Despite broad spectrum antibiotics and volume resuscitation, he had profound ischemia and impending gangrene of fingers, toes and nose. Heparin infusion had no effect on disease progression. Protein C activity was 61% and AT-III activity was 30%. Skin biopsy confirmed PF. Due to worsening multiple organ failure and progressive PF, AT-III infusion was initiated to target AT III levels to 120%. Plasma derived AT-III dose IU (120%-baseline % x Kg/1.4%) was administered for 72 hours. Target AT III was achieved on Day 4 by which time, the patient’s skin lesions, renal failure and shock resolved. He was extubated and had no bleeding complications. Purpuric lesions resolved over the next 2 weeks with no loss of digits except nose tip. He was discharged home. Discussion: Coagulopathy of DIC can manifest as bleeding or thrombosis due to an imbalance of pro and anti-coagulant factors. Severe microvascular thrombosis from consumption of protein C and AT -III as well as a complex interaction of endothelium, complement and inflammatory cytokines, is the hallmark of fatal septic PF/DIC. In thrombotic DIC, anticoagulation with heparin has mixed results. Our case illustrates that measurement of AT-III and protein C activity with targeted AT-III therapy may halt progression of heparin-unresponsive disease