Immunoadsorption with single-use columns for the management of bleeding in acquired hemophilia A: a series of nine cases.
BACKGROUND
Acquired hemophilia A in a setting of bleeding or required surgery frequently places patients into a state of critical illness with high mortality. In this context immunoadsorption (IA) can be used to eliminate coagulation inhibitors quickly to employ recombinant coagulation factors more effectively. However, since acquired hemophilia is a rare condition the therapy is little standardized.


METHODS
We report on a retrospective analysis of nine cases of acquired hemophilia A treated with IA using disposable adsorber columns. Data collection was performed by retrospectively reviewing the patients' files regarding clinical course, mode of therapy, inhibitor titers, and coagulation status.


RESULTS
Inhibitor titers were effectively reduced in all but one patient following the treatment with IA. In two out of seven patients surviving the acute bleeding an inhibitor relapse occurred. The overall remission rate was determined as 77.8% within a median follow-up of 613 days. In two of our nine patients fatal outcome resulted due to major bleeding complications. IA treatment showed good tolerability and no fatal complications were caused.


CONCLUSIONS
The presented cases support our assumption that patients with acquired hemophilia A benefit from IA with disposable columns in a setting of acute bleeding. This modality of IA is able to eliminate inhibitors reliably and quickly. IA in general is substantially speeding up the progress of therapy preventing bleeding complications constantly threatening the patient and reducing the dosages of coagulation factor therapy. We encourage IA with disposable columns in all bleeding patients with acquired hemophilia to aggressively lower the inhibitors.
Brzoska
Martin
M
Division of Nephrology, Department of Medicine, Johann Wolfgang Goethe-University, Frankfurt am Main, Germany. brzoska@em.uni-frankfurt.de
Krause
Manuela
M
Geiger
Helmut
H
Betz
Christoph
C
D000368:Aged; D003029:Coagulants / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D005260:Female; D006467:Hemophilia A / Q000150:complications*; D006470:Hemorrhage / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007163:Immunosorbent Techniques / Q000295:instrumentation; D008297:Male; D008875:Middle Aged
D003029:Coagulants