[Long-term remission after i.v. immunoglobulin therapy in acquired antihemophilic factor hemophilia with systemic lupus erythematosus].
A 27-year-old female with severe systemic lupus erythematosus with renal involvement developed extensive cutaneous hemorrhages 5 years after diagnosis. Routine coagulation tests confirmed a prolongation of activated partial thromboplastin time to 77 s. This was attributed to a marked reduction of factor VIII activity to less than 3%. An inhibitor with an activity of 1.4 Bethesda units against factor VIII was determined. Immunosuppressive therapy (steroids, azathioprin, cyclophosphamide, cyclosporine) had no influence on the hemorrhages. Later in the course of disease a life-threatening vaginal hemorrhage occurred in parallel with a flare-up of lupus activity. During that period a therapy of 7 S i.v. immunoglobulins (120 g within 5 days) was started. This led to an instant cessation of the bleeding. Factor-VIII activity rose from 3% ot 480% within 7 days and the ds-DNA-antibodies fell from 122 U/ml to 19.7 U/ml. Nine months later, under immunosuppressive therapy with cyclophosphamide and steroids, factor-VIII activity is still within the normal range and no bleeding episodes have occurred. This confirms the effectively of high-dose immunoglobulin therapy for hemophilia, due to acquired factor VIII antibodies, also in patients with severe SLE.
Pirner
K
K
Medizinische Klinik III mit Poliklinik, Friedrich-Alexander-Universität Erlangen-Nürnberg.
Rösler
W
W
Kalden
J R
JR
Manger
B
B
D000328:Adult; D001323:Autoantibodies / Q000032:analysis*; D005169:Factor VIII / Q000032:analysis / Q000276:immunology*; D005260:Female; D006467:Hemophilia A / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007116:Immunization, Passive / Q000379:methods*; D008180:Lupus Erythematosus, Systemic / Q000276:immunology / Q000628:therapy*; D008181:Lupus Nephritis / Q000276:immunology / Q000628:therapy; D014592:Uterine Hemorrhage / Q000276:immunology / Q000628:therapy
D001323:Autoantibodies; D005169:Factor VIII
Langzeitremission nach i.v. Immunglobulintherapie bei erworbener Hemmkörperhämophilie im Rahmen eines systemischen Lupus erythematodes.