RESUMO
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 a 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.
Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Autoanticorpos/imunologia , Doenças Autoimunes/tratamento farmacológico , Fator VIII/imunologia , Deficiência do Fator XI/tratamento farmacológico , Transtornos Puerperais/tratamento farmacológico , Hemorragia Uterina/etiologia , Adulto , Formação de Anticorpos/efeitos dos fármacos , Antígenos CD20/imunologia , Autoanticorpos/sangue , Doenças Autoimunes/etiologia , Doenças Autoimunes/imunologia , Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Cesárea , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Quimioterapia Combinada , Fator VIII/análise , Fator XI/análise , Fator XI/imunologia , Deficiência do Fator XI/imunologia , Feminino , Hematoma/etiologia , Hematoma/imunologia , Hemofilia A/tratamento farmacológico , Hemofilia A/imunologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Penfigoide Gestacional/imunologia , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Gravidez , Transtornos Puerperais/etiologia , Transtornos Puerperais/imunologia , Rituximab , Hemorragia Uterina/imunologiaRESUMO
OBJECTIVES: Rare patients with systemic lupus erythematosus (SLE) patients exhibit anticoagulants that interfere in the earlier stages of the intrinsic coagulation pathway, such as those involving factor XI (FXI). The objectives of our study were to describe the presence of an acquired inhibitor to FXI causing a life-threatening bleeding disorder in an SLE patient and to review the association of this coagulopathy with SLE. METHODS: We describe the clinical presentation of an SLE patient with an acquired FXI inhibitor. We reviewed the scientific literature using the MEDLINE database searching the following combinations of terms: "SLE and Factor XI," "SLE and Factor XI inhibitor," and "Factor XI inhibitor," from 1964 to 2007. RESULTS: A 20-year-old woman with a 6-year history of SLE was admitted to the hospital because of severe life-threatening abdominal bleeding due to a ruptured ovarian cyst. This hemorrhagic event was related to the presence of an FXI inhibitor. We reviewed another 13 SLE patients with this condition, 8 of whom had bleeding events. Most patients had manifestations of active SLE, and prednisone was used as the primary treatment. CONCLUSIONS: SLE activity seems to be associated with the production of antibodies directed against FXI, which may cause important coagulopathies, especially bleeding events. The inhibitor disappeared after immunosuppressive therapy for SLE in most cases, suggesting that the appearance of this inhibitor is immune mediated. Although the majority of cases with the FXI inhibitor are not fatal, it should be suspected and investigated in SLE patients, especially those with abnormal clotting tests.