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Abnormally large von Willebrand factor multimers in Henoch‐Schönlein purpura
Author(s) -
Casonato Alessandra,
Pontara Elena,
Bertomoro Antonella,
Ossi Elena,
Vincenti Massimo,
Girolami Antonio,
Borsatti Arturo,
Bertaglia Giselda
Publication year - 1996
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/(sici)1096-8652(199601)51:1<7::aid-ajh2>3.0.co;2-2
Subject(s) - purpura (gastropod) , henoch schonlein purpura , von willebrand factor , medicine , immunology , gastroenterology , vasculitis , platelet , biology , disease , ecology
Allergic vasculitis phenomena seem to be involved in Henoch‐Schönlein purpura (HSP). Elevated plasma levels of von Willebrand factor (vWf) are a well recognized feature of vasculitis and have been taken as an indication of in vivo endothelial cell damage. Plasma factor VIII:C and vWf levels and vWf multimeric pattern were studied in 8 patients with HSP, during active disease and twice during the remission (3 and 9 months later). Plasma vWf multimeric composition was evaluated using low resolution gels which better resolve large vWf multimers. During active disease plasma factor VIII:C, vWf:Ag, and vWf:RCoF were normal in 5% of patients and increased in three, but in each patient, platelets appeared to aggregate at doses of ristocetin lower than in normals. Furthermore, all patients demonstrated the presence of abnormally large vWf multimers usually found only in platelets and endothelial cells. Three and 9 months later, during remission, in spite of the normalization of factor VIII:C and vWf levels, the abnormal multimers were still detectable, as well as hyper‐responsiveness to ristocetin. These findings confirm that damage and/or perturbation of endothelial cells is associated with HSP. Moreover, the persistence of abnormality in the vWf multimeric pattern, when the disease is inactive, suggests that the mechanisms involved operate through the entire clinical course. © 1996 Wiley‐Liss, Inc.