Vol.22 No.5

Case Report

A case of catastrophic antiphospholipid antibody syndrome complicated with systemic lupus erythematosus, double positive for anti-cardiolipin/β2 glycoprotein I and anti-phosphatidylserine/ prothrombin autoantibodies

Authors

Eri Hirakawa1 , Kazuyoshi Saito1 , Shintaro Hirata1 , Tatsuya Atsumi2 , Takao Koike2 , Yoshiya Tanaka1

  • The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, 807-0855, Japan
  • Department of Medicine II, Hokkaido University Graduate School of Medicine, Sapparo, Japan
Received:

2 September 2011

Accepted:

11 November 2011

Published online:

29 November 2011

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Abstract

A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β2-glycoprotein I (β2GPI) were simultaneously detected, indicating that the different pathways of both PS/PT and CL/β2GPI might be associated with the radical manifestation of CAPS.

Key words

Anti-cardiolipin/b2-glycoprotein I (anti-CL/ β2GPI) antibody - Anti-phosphatidylserine/prothrombin (anti-PS/PT) antibody - Catastrophic antiphospholipid antibody syndrome (CAPS)