The relevance of the bleeding severity in the treatment of acquired haemophilia - an update of a single-centre experience with 67 patients.
Acquired haemophilia (AH), an autoimmune disorder with clinical features ranging from harmless haematomas to life-threatening bleedings, still has a mortality rate of up to 25%. Owing to its low frequency (1-4 x 10(6)), standardized treatment protocols for its variable manifestations are not available. In case of prominent severe bleedings, the treatment should aim at rapid elimination of the antibody to protect patients from bleedings and on reinduction of long-term immune tolerance. Clinical data, short- and long-term treatment results of 67 patients diagnosed by our centre are presented. Patients were treated depending on their bleeding severity either by an immunosuppressive treatment alone, or in case of life-threatening bleedings, by a combined protocol (modified Bonn-Malmö protocol, MBMP) consisting of antibody depletion through immunoadsorption, intravenous immunoglobulin treatment, immunosuppression and high-dose factor VIII (FVIII) substitution. Mild bleedings occurred in two patients who were treated successfully alone by immunosuppression. Complete remission (CR) was achieved in 90% of the patients treated with MBMP (60). Of the six patients (10%) who achieved a partial remission (PR), four suffered from cancer. Mortality under MBMP was not seen. In contrast, five patients, in whom diagnosis of AH was delayed, experienced fatal outcome during surgical interventions before initiation of MBMP treatment. Prognosis in AH depends mainly on its prompt diagnosis. Treatment procedures should be adapted to bleeding severity and inhibitor titres. Under these conditions, AH is a potentially curable autoimmune disorder with an excellent prognosis.
Zeitler
H
H
Internal Medical Clinic I of the University of Bonn, Centre of Extracorporeal Therapy and Autoimmunity (CETA,), Bonn, Germany. heike.zeitler@ukb.uni-bonn.de
Ulrich-Merzenich
G
G
Goldmann
G
G
Vidovic
N
N
Brackmann
H H
HH
Oldenburg
J
J
D000368:Aged; D019774:Blood Coagulation Factor Inhibitors / Q000032:analysis*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005169:Factor VIII / Q000008:administration & dosage*; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006467:Hemophilia A / Q000097:blood / Q000401:mortality / Q000628:therapy*; D006470:Hemorrhage / Q000517:prevention & control*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use
D019774:Blood Coagulation Factor Inhibitors; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D003520:Cyclophosphamide; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa