Several review articles aimed to discuss the etiology, diagnosis, and management of purpura fulminans (PF) to help guide clinical practice. Purpura fulminans is multifaceted, necessitating prompt diagnosis and comprehensive treatment strategies to curb the underlying cause and manage complex coagulopathy. Initial treatment includes supportive care and adequate hydration. Anticoagulation and replacement of blood, factors, and platelets may be required. Proposed treatments include Protein C (PC) concentrate, heparin, and therapeutic plasma exchange (TPE), all aimed at addressing the underlying coagulopathy. Ceprotin is available as the only PC concentrate in the United States. Cases of infectious PF require broad-spectrum antibiotics and consideration for intravenous immunoglobulin (IVIG) therapy. Medical management emphasizes the importance of timely intervention, leveraging a combination of anticoagulants and supportive therapies to mitigate the severe thrombotic and inflammatory manifestations of PF while data supporting the treatment options for PF are limited to small studies lacking robust methodology. [1], [2]
Children presenting with PF are initially assumed to have a septic cause and managed with full supportive care, urgent broad-spectrum antimicrobials, and adjunctive therapy. Fresh frozen plasma (FFP) is required in those with DIC, a potentially lethal complication of PF, at doses of 10–20 mL/kg every 8–12 hours. This intervention aims to replenish pro-coagulant and anticoagulant plasma proteins consumed during the DIC process. The treatment protocol may also include transfusions of platelet concentrates (10–15 mL/kg) or cryoprecipitate (5 mL/kg) for significant thrombocytopenia or hypofibrinogenemia, respectively. Empiric FFP is appropriate for other scenarios such as postinfectious PF and suspected severe heritable protein C (PC) or Protein S (PS) deficiency prior to definitive diagnosis. In scenarios where PF arises post-infection, the increased autoimmune clearance of PS might render FFP ineffective in achieving therapeutic plasma levels of protein S, prompting consideration for plasma exchange and immunosuppressive therapy. The role of Ceprotin in children with PF and severe sepsis remains unknown. Ceprotin has demonstrated safety and efficacy in the prevention of PF in severe heritable PC deficiency and may minimize FFP exposure. Anticoagulation should be used with caution but is usually necessary with acute PF with large vessel thrombosis and severe heritable PC deficiency. [3]
The challenge in treating PF underscores the urgency of its recognition and the rapid initiation of a comprehensive management strategy, balancing the aggressive replacement of depleted factors with meticulous attention to the potential for fluid overload and allergenic or immunologic responses to transfusions. The overarching goal in acute PF management is to prevent the progression of thrombotic injury while stabilizing or correcting DIC and preventing new lesions, adjusting the intensity of therapy based on the evolving clinical scenario and laboratory markers. The complexity of PF treatment, especially in children, stresses the necessity for careful consideration of the risks and benefits associated with specific therapeutic interventions, including the controversial use of recombinant PC concentrates in septic pediatric patients. Given the lack of large, well-designed clinical trials, future robust evidence is warranted to provide definitive guidance for the management of PF. [2], [3]