Acquired haemophilia A in women postpartum: management of bleeding episodes and natural history of the factor VIII inhibitor.
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.
Michiels
J J
JJ
Haematology, Haemostasis and Thrombosis Scientific Center, Goodheart Institute Rotterdam, The Netherlands.
Hamulyak
K
K
Nieuwenhuis
H K
HK
Novakova
I
I
van Vliet
H H
HH
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
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005169:Factor VIII; D011241:Prednisone