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Research Article| Volume 59, ISSUE 6, 102916, December 2020

Idiopathic TTP in the Middle East: Epidemiology and clinical outcomes in infection associated episodes

      Abstract

      Introduction

      Literature on epidemiology of thrombotic thrombocytopenic purpura (TTP) in the Middle East is scarce.

      Materials and methods

      We prospectively examined the association between infection and clinical outcomes in 44 patients with idiopathic TTP, with severely deficient ADAMTS13. We also investigated seasonality of the disease, hoping to better understand the epidemiology of idiopathic TTP.

      Results

      Summer demonstrated significantly lower incidence for idiopathic TTP, compared with other seasons P = 0.0003. Fourteen patients had 15 episodes with a suspected concomitant infection. Five initial episodes were triggered by an infection (33.3 %), all presenting in winter, six episodes were associated with an exacerbation (40 %) and infection triggered a relapse in the other four episodes (26.7 %), with 2 episodes presenting in winter.
      TTP associated infections included: central line infection, urinary tract infection and post-operative infection. One patient had respiratory tract infection, on both his initial and relapsing episodes. Refractoriness to treatment was demonstrated in 4 patients (28.6 %) and it was associated with dental abscess (one patient), septic shock (one patient) and Mycoplasma pneumonia (2 patients). All 4 patients had markedly elevated CRP values with a median of 335 mg/L.

      Conclusion

      Most of the infection associated episodes developed in winter (77.8 %).
      In patients with idiopathic TTP refractory to conventional treatment, infection should be seriously considered as an additional contributing factor for their initial and /or recurrent episodes, particularly when CRP is markedly elevated.

      Keywords

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