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[Complicated antiphospholid antibody syndrome].
F Langer1, B Eifrig, S Hegewisch-Becker
1Zentrum für Innere Medizin, Medizinische Klinik II, Onkologie und Hämatologie, Hamburg, Germany. drflanger@aol.com
Deutsche Medizinische Wochenschrift (1946)
|July 5, 2002
Summary
Antiphospholipid antibody syndrome (APAS) can be a life-threatening condition presenting with severe thromboembolism. Prompt diagnosis and individualized treatment, including anticoagulation and immunosuppression, are crucial for patient outcomes.
Area of Science:
- Rheumatology
- Hematology
- Internal Medicine
Background:
- Systemic lupus erythematosus (SLE) can lead to secondary antiphospholipid antibody syndrome (APAS), a prothrombotic condition.
- APAS is characterized by the presence of antiphospholipid antibodies, leading to an increased risk of venous and arterial thrombosis.
- Recurrent thromboembolic events despite standard anticoagulation necessitate a thorough investigation into underlying causes.
Observation:
- A 51-year-old female with invasive-ductal mamma carcinoma presented with recurrent ischemic strokes and venous thromboembolism.
- Clinical findings included hemiparesis, dysarthria, leg swelling, ankle ulceration, and a heart murmur.
- Laboratory results revealed anemia, thrombocytopenia, elevated inflammatory markers, and high titers of antinuclear, anticardiolipin, and anti-dsDNA antibodies, with a positive lupus anticoagulant test.
Findings:
- Deep vein thrombosis, mitral valve regurgitation, and pulmonary embolism were confirmed via imaging studies.
- The patient was diagnosed with systemic lupus erythematosus (SLE) and secondary antiphospholipid antibody syndrome (APAS).
- Initial treatment with ASS and phenprocoumon was insufficient, highlighting potential overestimation of oral anticoagulation effects due to lupus anticoagulant interference.
Implications:
- APAS can manifest as an acute, life-threatening disorder requiring urgent and specialized medical attention.
- Effective management involves a multidisciplinary approach and tailored treatment strategies, including switching to low-molecular-weight heparin and initiating immunosuppressive therapy (cyclophosphamide, azathioprine, prednisone).
- This case underscores the importance of recognizing APAS in patients with unexplained thromboembolism, particularly those with autoimmune conditions, and adjusting anticoagulation monitoring accordingly.