PMID- 34788405
OWN - NLM
STAT- MEDLINE
DCOM- 20220411
LR  - 20240410
IS  - 2473-9537 (Electronic)
IS  - 2473-9529 (Print)
IS  - 2473-9529 (Linking)
VI  - 6
IP  - 2
DP  - 2022 Jan 25
TI  - Idiopathic purpura fulminans associated with anti-protein S antibodies in 
      children: a multicenter case series and systematic review.
PG  - 495-502
LID - 10.1182/bloodadvances.2021005126 [doi]
AB  - 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.
CI  - (c) 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.
FAU - Theron, Alexandre
AU  - Theron A
AD  - Department of Pediatric Oncology and Hematology.
AD  - Resource and Competence Center for Hereditary Hemorrhagic Diseases, and.
FAU - Dautremay, Olivier
AU  - Dautremay O
AD  - Department of Biological Hematology, University of Montpellier, CHU Montpellier, 
      Montpellier, France.
AD  - Bioard'aisne Laboratory, Charleville-Mezieres, France.
FAU - Boissier, Elodie
AU  - Boissier E
AUID- ORCID: 0000-0002-7790-6375
AD  - Department of Biological Hematology, University of Montpellier, CHU Montpellier, 
      Montpellier, France.
AD  - Laboratory of Hematology, University Hospital, Nantes, France.
FAU - Zerroukhi, Amel
AU  - Zerroukhi A
AD  - Department of Pediatric Oncology and Hematology.
FAU - Baleine, Julien
AU  - Baleine J
AD  - Department of Neonatal Medicine and Pediatric Intensive Care, University of 
      Montpellier, CHU Montpellier, Montpellier, France.
FAU - Moulis, Lionel
AU  - Moulis L
AD  - Clinical Research and Epidemiology Unit, and.
FAU - Rodiere, Michel
AU  - Rodiere M
AD  - Department of Pediatrics, Infectious Diseases, and Immunology, University of 
      Montpellier, CHU Montpellier, Montpellier, France.
FAU - Schved, Jean-Francois
AU  - Schved JF
AUID- ORCID: 0000-0003-0332-3254
AD  - Resource and Competence Center for Hereditary Hemorrhagic Diseases, and.
AD  - Department of Biological Hematology, University of Montpellier, CHU Montpellier, 
      Montpellier, France.
FAU - Duraes, Martha
AU  - Duraes M
AUID- ORCID: 0000-0001-8126-7927
AD  - Anatomy Laboratory, University of Montpellier, Montpellier, France; and.
FAU - Kanouni, Tarik
AU  - Kanouni T
AD  - Department of Clinical Hematology, University of Montpellier, CHU Montpellier, 
      Montpellier, France.
FAU - Cau-Diaz, Isabelle
AU  - Cau-Diaz I
AD  - Department of Biological Hematology, University of Montpellier, CHU Montpellier, 
      Montpellier, France.
FAU - Jeziorski, Eric
AU  - Jeziorski E
AUID- ORCID: 0000-0003-0318-3044
AD  - Department of Pediatrics, Infectious Diseases, and Immunology, University of 
      Montpellier, CHU Montpellier, Montpellier, France.
FAU - Biron-Andreani, Christine
AU  - Biron-Andreani C
AUID- ORCID: 0000-0002-7549-8718
AD  - Resource and Competence Center for Hereditary Hemorrhagic Diseases, and.
AD  - Department of Biological Hematology, University of Montpellier, CHU Montpellier, 
      Montpellier, France.
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Systematic Review
PL  - United States
TA  - Blood Adv
JT  - Blood advances
JID - 101698425
RN  - 0 (Protein S)
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - Humans
MH  - *Chickenpox/complications
MH  - Delayed Diagnosis/adverse effects
MH  - Prospective Studies
MH  - Protein S
MH  - *Purpura Fulminans/diagnosis/etiology/therapy
MH  - Retrospective Studies
PMC - PMC8791598
EDAT- 2021/11/18 06:00
MHDA- 2022/04/12 06:00
PMCR- 2022/01/18
CRDT- 2021/11/17 17:28
PHST- 2021/04/30 00:00 [received]
PHST- 2021/10/21 00:00 [accepted]
PHST- 2021/11/18 06:00 [pubmed]
PHST- 2022/04/12 06:00 [medline]
PHST- 2021/11/17 17:28 [entrez]
PHST- 2022/01/18 00:00 [pmc-release]
AID - 482666 [pii]
AID - 2022/ADV2021005126 [pii]
AID - 10.1182/bloodadvances.2021005126 [doi]
PST - ppublish
SO  - Blood Adv. 2022 Jan 25;6(2):495-502. doi: 10.1182/bloodadvances.2021005126.