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Arch Pediatr. 2015 Nov;22(11):1167-70. doi: 10.1016/j.arcped.2015.07.014. Epub 2015 Sep 26.

[Mild hemophilia A fortuitously discovered during Henoch-Schönlein purpura].

[Article in French]

Author information

1
Service d'hématologie biologique, hôpital Antoine-Béclère, université Paris 11, Assistance publique-Hôpitaux de Paris, 157, avenue de la Porte-de-Trivaux, 92140 Clamart, France.
2
Centre régional de traitement de l'hémophilie et des maladies hémorragiques (CRTH), hôpital Bicêtre, université Paris 11, Assistance publique-Hôpitaux de Paris, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France.
3
Service d'hématologie biologique, hôpital Bicêtre, université Paris 11, Assistance publique-Hôpitaux de Paris, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France.
4
Service de pédiatrie générale, hôpital Antoine-Béclère, université Paris 11, Assistance publique-Hôpitaux de Paris, 157, avenue de la Porte-de-Trivaux, 92140 Clamart, France.
5
Service d'hématologie biologique, hôpital Antoine-Béclère, université Paris 11, Assistance publique-Hôpitaux de Paris, 157, avenue de la Porte-de-Trivaux, 92140 Clamart, France. Electronic address: agnes.veyradier@lrb.aphp.fr.

Abstract

Henoch-Schönlein 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.

PMID:
26412326
DOI:
10.1016/j.arcped.2015.07.014
[Indexed for MEDLINE]

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