Clinical heterogeneity of acquired hemophilia A: a description of 4 cases.
Acquired hemophilia A is a rare but severe auto-immune bleeding disorder characterized by the presence of autoantibodies directed against clotting factor VIII. Acquired hemophilia A may be idiopathic or associated with several conditions, such as postpartum, autoimmune diseases, malignancies or drugs. The treatment modalities of bleeding episodes and eradication of the factor VIII auto-antibody depend on the titer of anti-factor VIII:C and may include desmopressin (DDAVP), prednisolone, prednisolone-cyclophosphamide, high dose intravenous gammaglobulin, FVIII-VWF concentrate and/or recombinant FVIIa (rFVIIa). In this study we report four cases of autoimmune factor VIII inhibitors (2 associated with autoimmune disorders, 2 idiopathic) demonstrating the heterogeneity of this disease from pathogenic, clinical, therapeutic and prognostic points of view.
Franchini
Massimo
M
Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliera di Verona. massimo.franchini@mail.azosp.vr.it
Girelli
Domenico
D
Olivieri
Oliviero
O
Bozzini
Claudia
C
Guiotto
Maria
M
Zardini
Gabriele
G
Lippi
Giuseppe
G
Manzato
Franco
F
Gandin
Giorgio
G
D001323:Autoantibodies; D005169:Factor VIII / Q000276:immunology*; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology / Q000276:immunology*; D006801:Humans; D010641:Phenotype; D011379:Prognosis
D001323:Autoantibodies; D005169:Factor VIII