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Découverte fortuite d’une hémophilie A mineure à l’occasion d’un purpura rhumatoïde

Authors :
B. Joly
Roseline d'Oiron
L. Bendelac
A. Rafowicz
C. Meyzer
Agnès Veyradier
P. Labrune
C. Desconclois
Source :
Archives de Pédiatrie. 22:1167-1170
Publication Year :
2015
Publisher :
Elsevier BV, 2015.

Abstract

Henoch-Schonlein purpura is a common form of immunological vasculitis in children. Hemophilia A is a genetic disorder, inherited in a X-linked recessive pattern, and characterized by spontaneous hemorrhage or prolonged bleeding due to factor VIII deficiency. The clinical signs depend on the severity of factor VIII deficiency. We herein report the case of a 4-year-old boy admitted to the emergency room for typical rheumatoid purpura, associated with a lengthening of aPTT, whose exploration had uncovered mild hemophilia A. Laboratory assays should explore lengthening of aPTT: firstly the presence of lupus anticoagulant without bleeding risk, in an inflammatory context; secondly a deficiency of VWF and one of the factors involved in the extrinsic coagulation pathway associated with bleeding risk.

Details

ISSN :
0929693X
Volume :
22
Database :
OpenAIRE
Journal :
Archives de Pédiatrie
Accession number :
edsair.doi...........2ac557c1a5f907a8288ccbe1fb26b233
Full Text :
https://doi.org/10.1016/j.arcped.2015.07.014