Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review
| Autor: | Amel Zerroukhi, Martha Duraes, Julien Baleine, Jean-François Schved, Isabelle Cau-Diaz, Tarik Kanouni, Elodie Boissier, Olivier Dautremay, Lionel Moulis, Alexandre Theron, Eric Jeziorski, Christine Biron-Andreani, Michel Rodière |
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| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
medicine.medical_specialty
Delayed Diagnosis medicine.medical_treatment Werlhof's disease Gastroenterology Protein S Chickenpox Internal medicine medicine Humans Multicenter Studies as Topic Prospective Studies Child Coagulation Disorder Retrospective Studies biology business.industry Antithrombin Hematology respiratory system medicine.disease Stimulus Report humanities respiratory tract diseases body regions Platelet transfusion Child Preschool Purpura Fulminans biology.protein Plasmapheresis Fresh frozen plasma business medicine.drug |
| Zdroj: | Blood Advances |
| ISSN: | 2473-9537 2473-9529 |
| Popis: | Key Points IPF has a typical clinical presentation with involvement of the lower limbs corresponding to vascular territories.The antithrombin level and platelet count at diagnosis seemed to be associated with severe complications in IPF. Visual Abstract 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. |
| Databáze: | OpenAIRE |
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