Acquired haemophilia - a study of ten cases.
Clinico-haematological features in 10 patients with acquired Haemophilia are 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 coexisted with Factor VIII inhibitors in three patients. All patients presented with sudden onset of bleeding without any past or family history of a bleeding disorder. Factor VIII inhibitor levels ranged from 8 to 512 Bethesda units in the nine patients. Immunosuppressive therapy was given to 8 patients, consisting of CVP regimen or corticosteroids with endoxan or cyclosporin. Seven 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 Haemophilia, postpartum Haemophilia has good prognosis.
Saxena
R
R
Department of Haematology, All India Institute of Medical Sciences, New Delhi, India.
Mishra
D K
DK
Kashyap
R
R
Choudhry
V P
VP
Mahapatra
M
M
Bhargava
M
M
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D001779:Blood Coagulation Factors / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D016572:Cyclosporine / Q000008:administration & dosage; D003937:Diagnosis, Differential; D019468:Disease Management; D005169:Factor VIII / Q000276:immunology; D005192:Family Health; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology; D006470:Hemorrhage / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007194:India; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D008875:Middle Aged; D049590:Postpartum Period / Q000097:blood / Q000276:immunology; D011241:Prednisone / Q000008:administration & dosage; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000188:drug therapy; D012771:Shock, Hemorrhagic / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D001779:Blood Coagulation Factors; D007166:Immunosuppressive Agents; D016682:Lupus Coagulation Inhibitor; D014750:Vincristine; D016572:Cyclosporine; D003520:Cyclophosphamide; D005169:Factor VIII; C065655:anti-inhibitor coagulant complex; D011241:Prednisone