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137 A case of catastrophic antiphospholipid syndrome was controlled with rituximab
  1. KN Kim1,
  2. S Lee1 and
  3. JW Rhim2
  1. 1Hallym University Sacred Heart Hospital, Paediatrics, Anyang- Gyeonggi-do, Republic of Korea
  2. 2The Catholic University, Paediatrics, Daejeon, Republic of Korea

Abstract

Background and Aims CAPLS is a multiple thrombi of small vessels affecting viscera. Patients had previous evidence of APLS. CAPLS was precipitated by infection, invasive procedures. The clinical presentation was involving the cardiopulmonary, CNS, abdomem. Thrombocytopenia and hemolytic anaemia were frequent. Anticardiolipin antibodies were present in almost. The outcome can be disastrous.

Methods Case: 10-year-girl had been developed petechiae on Aug 10, 2009. Patelet 2000, Hb 12.0. The diagnosis was ITP and she received the IVIG. But anaemia was developed on Aug 31, 2009. Platelet 48,000. Hb 8.9. The diagnosis was Acquired hemolytic anaemia. On Sept 21, 2009 the Hb 10.0, reticulocyte 11.0% and Cooms’ (+), FANA (++), anti-dsDNA (-). The diagnosis was Evans’ syndrome. The following lab were anti-cardiolipin IgG (+), IgM (+), IgA (-), Lupus anticoagulant (+), anti-beta2 GPI IgG (+), IgM (+) on Oct 14, 2009. The final diagnosis was SLE with antiphosolipid syndrome.

Gangrene affecting the right 5th toe on Jan 2014 and gangrened toe was autoamputated on Dec 2014. CT angiogram was performed on Apr 2014. There was superficial occusion on right femoral vein. On July 2014 the disease was flared. Hemolytic anaemia (Hb 7.7) and jaundice developed. Chest CT revealed SLE myocarditis and abdomen CT was bowel SLE.

Results On Sept 2014, catastrophic APS was developed. Platelet 5,000. aPTT 99.3 s. Plasma pheresis was done for 7 times, but the platelet count did not change. Rituximab was injected 4 times. Platelet was 4000 thru 99 000 on Nov 2014. After then platelet was well controlled.

Conclusions This rituximab response to therapy.

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