title: [Long-term response to rituximab in a patient with acquired hemophilia].
abstract: 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.
authors: Jaime
authors: J
authors: García-Chávez
authors: Jorge
authors: J
authors: Vela-Ojeda
authors: Aurora
authors: A
authors: García-Manzano
authors: Abraham
authors: A
authors: Majluf-Cruz
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000917:Antibody Formation / Q000187:drug effects; D018951:Antigens, CD20 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D001402:B-Lymphocytes / Q000187:drug effects / Q000276:immunology; D002585:Cesarean Section; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005169:Factor VIII / Q000032:analysis / Q000276:immunology*; D005172:Factor XI / Q000032:analysis / Q000276:immunology; D005173:Factor XI Deficiency / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D006406:Hematoma / Q000209:etiology / Q000276:immunology; D006467:Hemophilia A / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D006559:Pemphigoid Gestationis / Q000276:immunology; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D011247:Pregnancy; D011644:Puerperal Disorders / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D000069283:Rituximab; D014592:Uterine Hemorrhage / Q000209:etiology* / Q000276:immunology
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide; D005169:Factor VIII; D005172:Factor XI; D011241:Prednisone; D008775:Methylprednisolone