Abstract |
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.
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Authors | K Pirner, W Rösler, J R Kalden, B Manger |
Journal | Zeitschrift fur Rheumatologie
(Z Rheumatol)
1990 Nov-Dec
Vol. 49
Issue 6
Pg. 378-81
ISSN: 0340-1855 [Print] Germany |
Vernacular Title | Langzeitremission nach i.v. Immunglobulintherapie bei erworbener Hemmkörperhämophilie im Rahmen eines systemischen Lupus erythematodes. |
PMID | 2128157
(Publication Type: Case Reports, English Abstract, Journal Article)
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Chemical References |
- Autoantibodies
- Factor VIII
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Topics |
- Adult
- Autoantibodies
(analysis)
- Factor VIII
(analysis, immunology)
- Female
- Hemophilia A
(immunology, therapy)
- Humans
- Immunization, Passive
(methods)
- Lupus Erythematosus, Systemic
(immunology, therapy)
- Lupus Nephritis
(immunology, therapy)
- Uterine Hemorrhage
(immunology, therapy)
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