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Long-term efficacy of immunoadsorbent plasmapheresis in a patient with Budd-Chiari syndrome due to antiphospholipid syndrome: case report with nine-year follow-up

Molecular Genetics of Cardiovascular Disorders, Graduate School of Medical Science, Kanazawa University, Japan, diabe{at}med.kanazawa-u.ac.jp

M Kawano

Molecular Genetics of Cardiovascular Disorders, Graduate School of Medical Science, Kanazawa University, Japan

T Haraki

Komatsu Municipal Hospital, Komatsu, Japan

T Higashikata

Molecular Genetics of Cardiovascular Disorders, Graduate School of Medical Science, Kanazawa University, Japan

K Ueda

Komatsu Municipal Hospital, Komatsu, Japan

T Okada

Molecular Genetics of Cardiovascular Disorders, Graduate School of Medical Science, Kanazawa University, Japan

I Koni

Molecular Genetics of Cardiovascular Disorders, Graduate School of Medical Science, Kanazawa University, Japan

H Mabuchi

Molecular Genetics of Cardiovascular Disorders, Graduate School of Medical Science, Kanazawa University, Japan

We describe the safety and efficacy of long-term immunoadsorbent plasmapheresis (IAPP) with dextran sulfate-cellulosebead columnsin antiphospholipidsyndrome (APS). IAPP was administeredto a 38-year old male Japanese patient with APS with Budd-Chiari syndrome (BCS), who had presented with refractory lower leg skin ulcers and arterial and venous thromboses including BCS. After hepatic vein transluminal angioplasty was performed, the combination of corticosteroid, aspirin and IAPP was administeredbecause of an underlyingbleedingtendencyrelated to liver dysfunction.From February 1994 to February 2003, a total of 228 procedureswere performed. No further thrombosis-relatedsymptoms or bleeding have occurred for more than nine years, suggesting that IAPP with dextran sulfate-cellulose columns is safe and effective for APS in preventingadditional thrombotic events. This IAPP supplements anticoagulation, antiplatelet, corticosteroid and immunosuppressant therapies.

Key Words: antiphospholipidsyndrome • Budd-Chiari syndrome • immunoadsorbent plasmapheresis

Lupus, Vol. 13, No. 2, 135-138 (2004)
DOI: 10.1191/0961203304lu486cr


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