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A 28 year-old female without history of previous disease. In the seventh month of her first pregnancy she developed hemorrhagic tendency that worsened in the early postpartum period. Activated partial thromboplastin time was 110 sec (control = 35.8 sec) with negative tests for lupus anticoagulant. Factor VIII was < 1% and a factor VIII inhibitor titer was 84 Bethesda Units/mL (BU). Initial therapy included methylprednisolone, prednisone, and cyclophosphamide. After two weeks of treatment, clinical conditions of the patient improved slightly and she was discharged. Outpatient therapy included azathioprine, and prednisone for a period of 22 months but in-hospital management was several times required. We initiated rituximab 375 mg/m²/week/4 weeks. A clinical improvement and increased levels of factors VIII and XI were observed 10 weeks later and factor VIII inhibitor decreased to undetectable levels. After an 82-month follow-up period (since the first rituximab infusion), she is asymptomatic and factor VIII and factor XI plasma levels are 70 and 94%, respectively. FVIII inhibitor level is still undetectable. Rituximab seems an alternative for the treatment of acquired hemophilia refractory to standard treatment.

Palabras clave: Hemophilia. Acquired hemophilia. Rituximab. Factor VIII. Factor IX.

2013-07-24   |   627 visitas   |   Evalua este artículo 0 valoraciones

Vol. 63 Núm.2. Marzo-Abril 2011 Pags. 210-212 Rev Invest Clin 2011; 63(2-ENGLISH)