Post-viral idiopathic purpura fulminans is associated with inherited thrombophilia and anti-cardiolipin antibodies.
Front Pediatr
; 11: 1197795, 2023.
Article
en En
| MEDLINE
| ID: mdl-37325350
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 (FIIG20210A),Factor V R506Q polymorphism (FVR506Q); and/or for APL (lupus anticoagulant (LA), anti-cardiolipin antibodies (ACL), or anti-beta 2-GPI antibodies (Aß2GP1).Results:
Among the 25 patients tested for inherited thrombophilia, 7 (28%) had positive results. Three had FV R506Q, two FIIG20210A, one compound heterozygote FVR506Q associated to FIIG20210A, 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 Aß2GP1 (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.
Texto completo:
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Base de datos:
MEDLINE
Tipo de estudio:
Clinical_trials
/
Risk_factors_studies
Idioma:
En
Revista:
Front Pediatr
Año:
2023
Tipo del documento:
Article
País de afiliación:
Francia