2002 article

Acquired type 3-like von Willebrand syndrome preceded full-blown systemic lupus erythematosus

Masami Niiya, K Niiya, Yuki Takazawa, Yuichiro Hayashi, Yoshiro Tanio, Masahiko Kushiro, Masahito Tanimizu, Harumi Hasegawa, Mitsuo Tanimoto

20Citations signalées, ce qui n’est pas une note de qualité

2Institutions déclarées

1Pays d’affiliation déclarés

Rattachement africain : jp. Niveau de preuve : code pays fourni par la source.

Le résumé fourni par la source

We report a quite rare case of acquired type 3-like von Willebrand syndrome (vWS) that preceded full-blown systemic lupus erythematosus (SLE). A 16-year-old woman with no previous disease history and no family history of hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis and gingival bleeding. She was diagnosed as having atypical type 3 von Willebrand disease because of prolonged bleeding time with normal platelet count and prolonged activated partial thromboplastin time (aPTT), and an almost complete absence of von Willebrand factor (vWF) antigen, ristocetin cofactor activity (vWF:RCo) and ristocetin-induced platelet agglutination (RIPA). Furthermore, electrophoretic analysis of plasma vWF revealed a trace amount of vWF and an absence of the multimeric form of vWF. Infusions of either vasopressin or factor VIII/vWF concentrates improved bleeding symptoms and corrected the aPTT and RIPA. However, she complained of low-grade fever, general fatigue and polyarthralgia 5 months later, and leukocytepenia and hypo-complementemia developed. Anti-double-stranded DNA antibodies and lupus erythematosus cells became positive. These findings were compatible with SLE. Mixing the patient's platelet-poor plasma (PPP) with normal platelet-rich plasma (PRP) (PPP/PRP = 2/1) resulted in a complete inhibition of RIPA, suggesting the presence of vWF inhibitor in her plasma. Treatment with prednisolone (40 mg/day) started and the bleeding tendency gradually improved. One month later, all of the laboratory data including aPTT, bleeding time, RIPA and vWF:RCo became normal. These findings indicate that she has an acquired type 3-like vWS associated with SLE.

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Le contrôle bibliographique ouvert

DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.

Titre Crossref: Acquired type 3-like von Willebrand syndrome preceded full-blown systemic lupus erythematosus
Date Crossref: 01/06/2002
Éditeur: Ovid Technologies (Wolters Kluwer Health)
Type: journal-article

Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.

Où se fait cette recherche

Okayama University Second Department of Medicine pays non établi dans la notice
Université ou école supérieure
Tottori Municipal Hospital pays non établi dans la notice
Établissement de santé
(Received 1 November 2001 pays non établi dans la notice
Institution
and Y. Hayashi and Y. Tanio are with the Central Laboratory M. Tanimizu and H. Hasegawa are with the Department of Internal Medicine pays non établi dans la notice
Structure de recherche
accepted 4 January 2002) pays non établi dans la notice
Institution
e-mail: [email protected] pays non établi dans la notice
Institution
fax: (+81) 86 232 8226 pays non établi dans la notice
Institution
revised 2 January 2002 pays non établi dans la notice
Institution

Second Department of Medicine — Okayama University, Tottori Municipal Hospital et (Received 1 November 2001, avec 5 autres affiliations.

Une affiliation ne permet pas de déduire la nationalité d’un auteur.

Les sujets associés

Platelet Disorders and TreatmentsBlood groups and transfusionHeparin-Induced Thrombocytopenia and Thrombosis

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