Acquired haemophilia - a study of ten cases

Clinico-haematological features in 10 patients with acquired Haemophilia ar e presented. Three patients had FVIII inhibitors following pregnancy while in six the cause for development of inhibitors could not be determined. One patient had acquired von Willebrand's disease. Lupus anticoagulant coexist ed with Factor VIII inhibitors in three patients. All patients presented wi th sudden onset of bleeding without any past or family history of a bleedin g disorder. Factor VIII inhibitor levels ranged from 8 to 512 Bethesda unit s in the nine patients. Immunosuppressive therapy was given to 8 patients, consisting of CVP regimen or corticosteroids with endoxan or cyclosporin. S even patients had clinical and laboratory responses and one patient did not respond. One patient had severe postpartum bleeding with acute shock which was controlled with FEIBA. Diagnosis and management of idiopathic acquired Haemophilia, thus, continues to be a major challenge, and among acquired H aemophilia, postpartum Haemophilia has good prognosis.