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Successful treatment of a case of catastrophic antiphospholipid syndrome with autologous BMT: case report and review of literature

Bone Marrow Transplantation volume 46, pages 597–600 (2011)Cite this article

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Antiphospholipid syndrome (APS) is an auto-Ab-mediated prothrombotic disorder characterized by recurrent venous and arterial thromboembolic events. It is defined by the Sapporo criteria as the presence of antiphospholipid Abs, either lupus anticoagulant (LA), anticardiolipin antibodies (ACAs) or anti-β2-glycoprotein Abs (β2GP1) with occurrence of either thromboembolic events or pregnancy morbidity.1 Administration of anticoagulant and anti-platelet agents is the standard therapeutic intervention for patients who develop thromboembolic manifestation. Some patients show more serious complications with a very high auto-Ab titer and with failure of anticoagulant and anti-platelet agents to prevent thrombosis. These patients may require immunomodulation to control the disease.2, 3

A 34-year-old male was diagnosed as having primary APS fulfilling the Sapporo criteria. He first presented at the age of 25 years with deep venous thrombosis. He was treated with standard anticoagulation therapy and maintained at an INR of 2–3. Over 3 years, he had five additional episodes of deep venous thrombosis/pulmonary embolism, with peripheral ischemia and suspicion of acute myocardial infarction. He was referred to our institution for further management and for cardiac catheterization. However, because of extensive thrombosis, cardiac catheterization could not be performed, and he was continued on warfarin at the same dose in addition to aspirin 325 mg. Initial workup showed the presence of strong LA with an LA ratio of 4.2 (positive >1.35), ACA IgG was 633 IgG phospholipid (GPL)/mL, β2GP1 IgG was 928 GPL/mL and both ANA and dsDNA were negative. He was positive for activated protein C resistant ratio. All other thrombophilia tests, including molecular studies for factor V Leiden and prothrombin 20210 mutations were negative. Complete blood count, liver function and renal functions were all within normal ranges. The patient was switched to low molecular weight heparin and monitored with the intention of giving anti-Xa of 0.5–1.0 U/mL because of the difficulty of maintaining him at a therapeutic INR.

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Acknowledgements

We thank Dr T Ortel and Dr M Crowther for their valuable advice during the management of this patient.

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Authors and Affiliations

  1. Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia

    T M Owaidah

  2. Intensive Care Department, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia

    K Maghrabi

  3. Medicine Department, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia

    T M Owaidah, M A Elkarouri & A Al Harthi

  4. Oncology Centre, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia

    F Al Mohareeb & H Al Zahrani

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  1. T M Owaidah
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Correspondence to T M Owaidah.

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Owaidah, T., Maghrabi, K., Elkarouri, M. et al. Successful treatment of a case of catastrophic antiphospholipid syndrome with autologous BMT: case report and review of literature. Bone Marrow Transplant 46, 597–600 (2011). https://doi.org/10.1038/bmt.2010.148

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