Post-viral idiopathic purpura fulminans is associated with inherited thrombophilia and anti-cardiolipin antibodies

A Theron; S Ayadi; E Boissier; O Dautremay; J-F Schved; N Sirvent; I Diaz; G Captier; C Biron-Andreani; E Jeziorski

doi:10.3389/fped.2023.1197795

Post-viral idiopathic purpura fulminans is associated with inherited thrombophilia and anti-cardiolipin antibodies

Front Pediatr. 2023 May 30:11:1197795. doi: 10.3389/fped.2023.1197795. eCollection 2023.

Authors

A Theron^{1

2

3}, S Ayadi¹, E Boissier⁴, O Dautremay^{5

6}, J-F Schved⁶, N Sirvent¹, I Diaz⁶, G Captier^{7

8}, C Biron-Andreani^{2

6}, E Jeziorski^{9

10}

Affiliations

¹ Department of Pediatric Oncology and Hematology, CHU de Montpellier, University of Montpellier, Montpellier, France.
² Hemophilia Treatment Center, Montpellier, France.
³ IRMB, University of Montpellier, INSERM, Montpellier, France.
⁴ Laboratory of Hematology, University Hospital, Nantes, France.
⁵ Biology Laboratory, Charleville-Mézières, France.
⁶ Department of Biological Hematology, CHU de Montpellier, University of Montpellier, Montpellier, France.
⁷ Department of Plastic, Reconstructive and Aesthetic Surgery, CHU de Montpellier, University of Montpellier, Montpellier, France.
⁸ LIRMM, CNRS-University of Montpellier, Montpellier, France.
⁹ Department of Pediatric Infectious Diseases and Immunology, CHU de Montpellier, University of Montpellier, Montpellier, France.
¹⁰ PCCEI, CeRéMAIA, CHU de Montpellier, University Montpellier, Montpellier, France.

Abstract

Introduction: Idiopathic purpura fulminans (IPF) is a rare and severe coagulation disorder, associated with transient anti-protein S (anti-PS) antibodies in the context of post-viral infection such as varicella. Anti-protein S antibodies are frequently found in the context of varicella, in contrast with the rarity of IPF. Other factors such as anti-phospholipid antibodies (APL) and inherited thrombophilia may be associated with severe vascular complication.

Method: This is an ancillary study of a French multicenter retrospective series and systematic review of literature. We analyzed patients who were tested for inherited thrombophilia, namely antithrombin, protein C, protein S deficiency; prothrombin gene G20210A polymorphism (FII:G20210A),Factor V R506Q polymorphism (FV:R506Q); and/or for APL (lupus anticoagulant (LA), anti-cardiolipin antibodies (ACL), or anti-beta 2-GPI antibodies (Aβ2GP1).

Results: Among the 25 patients tested for inherited thrombophilia, 7 (28%) had positive results. Three had FV R506Q, two FII:G20210A, one compound heterozygote FV:R506Q associated to FII:G20210A, and one protein C deficiency. APL testing was performed in 32 patients. It was positive in 19 patients (59%): 17 ACL (53%), 5 LA (16%), 4 Aβ2GP1 (13%). The risk of severe complications was not associated with presence of inherited thrombophilia or APL presence, with RR: 0.8 [95% CI: 0.37-1.71], p = 1 and RR: 0.7 [95% CI: 0.33-1.51], p = 0.39, respectively. We found a high prevalence of inherited thrombophilia or APL in a population of patients with IPF. However, we do not find an association with the occurrence of severe vascular complications or venous thromboembolism.

Keywords: anti-cardiolipin antibodies; chickenpox; child; purpura fulminans; thrombophilia.