[The diagnosis and therapy of acquired factor-VIII inhibitors in combination with lupus anticoagulants].
METHODS
A 63-year-old woman, known to have a primary factor VIII inhibitor (FFI) in combination with lupus anticoagulants (LA) was hospitalised because of life-threatening bleeding from mouth and neck.


METHODS
The activity of coagulation factor VIII was 9% under substitution, while the factor VIII inhibitor titre was 123 U/ml. A lupus anticoagulant test was positive. Antibodies against varicella-zoster virus and Epstein-Barr virus were demonstrated. The right adrenal was found to be enlarged on computed tomography.


METHODS
Coagulation became normal on administration of porcine factor VIII concentrate. Three cycles of a combination of three protein A immunoadsorptions, cyclophosphamide (twice 1.0 g intravenously), IgG (30 g daily for 5 days) as well as long-term oral cyclophosphamide administration (150 mg daily) during the interval were undertaken to reduce the inhibitor and produce immuno-tolerance. The factor VIII inhibitor titre was stabilised at a low level, but factor VIII activity could not be normalised without substitution.


CONCLUSIONS
The simultaneous presence of specific and non-specific inhibitors makes laboratory diagnosis and treatment more difficult. Porcine factor VIII and a combination of immunoadsorption and suppression are important components in the treatment of bleeding episodes and the production of immunotolerance.
Grossmann
R
R
Zentrallabor, Medizinische Universitätsklinik, Würzburg.
Wankmüller
H
H
Schwender
S
S
Kirschner
J
J
Mansouri-Taleghani
B
B
Keller
P
P
Kochsiek
K
K
D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005169:Factor VIII / Q000032:analysis / Q000037:antagonists & inhibitors*; D005260:Female; D006470:Hemorrhage / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D006801:Humans; D007108:Immune Tolerance; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D008875:Middle Aged; D009059:Mouth Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D009333:Neck
D016682:Lupus Coagulation Inhibitor; D005169:Factor VIII
Diagnose und Therapie eines erworbenen Faktor-VIII-Inhibitors in Kombination mit Lupus-Antikoagulanzien.