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Steady improvement of prothrombin levels after cyclophosphamide therapy in pediatric lupus anticoagulant hypoprothrombinemia syndrome (LAHPS)
- 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 :
- Male
medicine.medical_specialty
Adolescent
Cyclophosphamide
Azathioprine
Gastroenterology
factor II deficiency
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS)
systemic lupus erythematosus (SLE)
Rheumatology
Internal medicine
medicine
Humans
Lupus Erythematosus, Systemic
Child
skin and connective tissue diseases
Hypoprothrombinemias
Lupus anticoagulant
biology
business.industry
Syndrome
General Medicine
Mixing study
medicine.disease
Immunology
biology.protein
Female
Prothrombin
Antibody
Hypoprothrombinemia
business
Biomarkers
Immunosuppressive Agents
Follow-Up Studies
medicine.drug
Anti-SSA/Ro autoantibodies
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....60ae7624085c26301cc50efed3c5b270