Recurrent thrombotic thrombocytopenicpurpura in systemic lupus erythematosus patient

Authors: Z. Fojtík;  Z. Kořístek;  M. Klabusay;  M. Navrátil
Authors‘ workplace: Interní hematoonkologická klinika, FN Brno Bohunice
Published in: Čes. Revmatol., , 2003, No. 3, p. 157-160.


The onset of thrombotic thrombocytopenic purpura (TTP) in a patient followed up for SLE isdescribed. TTP is assigned to the thrombocytopenias with high turnover and peripheral plateletdestruction. Aetiopathogenesis of TTP seems to be caused by structural alteration of vonWillebrandfactor (vWF), resulting in the high multimers (ultra large–UL) presence. Treatment consists of vWFmetalloproteinase administration by fresh frozen plasma or plasma exchange by plasmapheresis.The case of 35 years old woman with TTP followed up for SLE is described. She was admitted withhaemolytic anaemia with later evolution of neurological symptoms and respiratory arrest due topulmonary oedema necessitating ventilatory support and with manifestation of cardiac insufficiency.The presence of schistocytes, thrombocytopenia, fever, and neurological symptoms led to thediagnosis of TTP. Treatment using high doses of fresh frozen plasma or plasma exchange byplasmapheresiswasstartedandpromptimprovement of clinicalandlaboratory statuswasobserved.Elective splenectomy was performed for TTP relaps which resulted again in the improvement ofblood count. TTP can occur as secondary syndrom in systemic connective tissue diseases or canoriginate in consequence of some drug treatment. It is necessary to think of the disease in case ofdecline of blood count in such conditions, since not recognized or inadequate treatment can lead tolethal outcome.

Key words:
SLE, thrombotic thrombocytopenic purpura

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Dermatology & STDs Paediatric rheumatology Rheumatology
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