Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review.

Blood advances 2022 Jan 25

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Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic coagulation disorder that can occur after chickenpox or human herpesvirus 6 (HHV-6) infection. IPF leads to an autoantibody-mediated decrease in the plasma concentration of protein S. We conducted a retrospective multicenter study involving patients with IPF from 13 French pediatric centers and a systematic review of cases in published literature. Eighteen patients were included in our case series, and 34 patients were included as literature review cases. The median age was 4.9 years, and the diagnostic delay after the first signs of viral infection was 7 days. The lower limbs were involved in 49 patients (94%) with typical lesions. In all, 41 patients (78%) had a recent history of varicella-zoster virus infection, and 7 patients (14%) had been infected by HHV-6. Most of the patients received heparin (n = 51; 98%) and fresh frozen plasma transfusions (n = 41; 79%); other treatment options were immunoglobulin infusion, platelet transfusion, corticosteroid therapy, plasmapheresis, and coagulation regulator concentrate infusion. The antithrombin level and platelet count at diagnosis seemed to be associated with severe complications. Given the rarity of this disease, the creation of a prospective international registry is required to consolidate these findings. © 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.

Citation

Alexandre Theron, Olivier Dautremay, Elodie Boissier, Amel Zerroukhi, Julien Baleine, Lionel Moulis, Michel Rodière, Jean-François Schved, Martha Duraes, Tarik Kanouni, Isabelle Cau-Diaz, Eric Jeziorski, Christine Biron-Andreani. Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review. Blood advances. 2022 Jan 25;6(2):495-502