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Steady improvement of prothrombin levels after cyclophosphamide therapy in pediatric lupus anticoagulant hypoprothrombinemia syndrome (LAHPS).
- Source :
-
Clinical rheumatology [Clin Rheumatol] 2007 Dec; Vol. 26 (12), pp. 2167-2169. Date of Electronic Publication: 2007 May 22. - Publication Year :
- 2007
-
Abstract
- Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare acquired disorder associated with several different conditions but mostly with systemic lupus erythematosus (SLE). LAHPS probably results from the presence of anti-Factor II antibodies, which usually counterbalance the prothrombotic effect of the lupus anticoagulant (LAC). In fact, Factor II deficiency in SLE is invariably associated with the presence of LAC. No consensus exists for the treatment of LAHPS. Corticosteroids, with or without the addition of vitamin K or blood products, have been a successful first-line treatment. Immunoglobulin (IVIG) treatment has been shown to be effective in the setting of acute bleeding. However, in some patients, conservative treatment is not enough to control bleeding, and the addition of immunosuppressive therapy, usually azathioprine, is needed. In our patients, Factor II deficiency reappeared after tapering steroids. Both children achieved normal Factor II levels with cyclophosphamide. This effect was long-lasting, a phenomenon that has not been documented in children prior to this report.
- Subjects :
- Adolescent
Biomarkers blood
Child
Female
Follow-Up Studies
Humans
Hypoprothrombinemias drug therapy
Hypoprothrombinemias etiology
Lupus Erythematosus, Systemic blood
Lupus Erythematosus, Systemic complications
Male
Syndrome
Cyclophosphamide therapeutic use
Hypoprothrombinemias blood
Immunosuppressive Agents therapeutic use
Lupus Erythematosus, Systemic drug therapy
Prothrombin metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0770-3198
- Volume :
- 26
- Issue :
- 12
- Database :
- MEDLINE
- Journal :
- Clinical rheumatology
- Publication Type :
- Academic Journal
- Accession number :
- 17516128
- Full Text :
- https://doi.org/10.1007/s10067-007-0647-5