title: Acquired haemophilia A in women postpartum: management of bleeding episodes and natural history of the factor VIII inhibitor.
abstract: The present study reports on the treatment of bleeding episodes and the natural history of factor VIII inhibitors in 4 patients with acquired haemophilia A postpartum. Low titre type II factor VIII inhibitors in 3 patients and high titre type I inhibitor in 1 patient became apparent immediately to 7 months after delivery. High dose human factor VIII concentrate substitution was effective in controlling bleeding episodes in two cases of factor VIII inhibitor type II, but ineffective in 1 patient with high titre type I factor VIII inhibitor. High dose gammaglobulin intravenously in 1 patient with type II factor VIII inhibitor induced a partial correction of factor VIIIc levels for 2 wk. Immunosuppressive treatment in all 4 patients with acquired haemophilia A postpartum did not reduce the potency of the factor VIII inhibitors. The low titre type II inhibitors spontaneously disappeared in all 3 patients within a few months to 1 yr after discontinuation of the immunosuppressive treatment. The high titre type I factor VIII inhibitor persisted for more than 24 yr. We conclude that immunosuppression in 4 women with acquired haemophilia A postpartum did not significantly affect the factor VIII inhibitor titre.
authors.affiliation: Haematology, Haemostasis and Thrombosis Scientific Center, Goodheart Institute Rotterdam, The Netherlands.
authors.forename: J J
authors.initials: JJ
authors.lastname: Michiels
authors.forename: K
authors.initials: K
authors.lastname: Hamulyak
authors.forename: H K
authors.initials: HK
authors.lastname: Nieuwenhuis
authors.forename: I
authors.initials: I
authors.lastname: Novakova
authors.forename: H H
authors.initials: HH
authors.lastname: van Vliet
mesh_terms: D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology* / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D049590:Postpartum Period; D011241:Prednisone / Q000627:therapeutic use; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic; D013997:Time Factors
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005169:Factor VIII; D011241:Prednisone