Department of Clinical Pathology, Division of Transfusion Medicine and Blood Banking, Cairo University, Cairo, Egypt.
Transfus Apher Sci. 2020 Dec;59(6):102916. doi: 10.1016/j.transci.2020.102916. Epub 2020 Aug 26.
Literature on epidemiology of thrombotic thrombocytopenic purpura (TTP) in the Middle East is scarce.
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.
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.
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.
中东地区关于血栓性血小板减少性紫癜（TTP）的流行病学文献很少。
我们前瞻性地研究了 44 例严重缺乏 ADAMTS13 的特发性 TTP 患者感染与临床结局之间的关系。我们还调查了疾病的季节性，希望更好地了解特发性 TTP 的流行病学。
与其他季节相比，夏季特发性 TTP 的发病率明显较低（P=0.0003）。14 例患者有 15 次疑似同时感染。5 次初始发作由感染引发（33.3%），均发生在冬季，6 次与恶化相关（40%），另外 4 次由感染引发复发（26.7%），其中 2 次发生在冬季。TTP 相关感染包括：中心静脉导管感染、尿路感染和术后感染。1 例患者在初始和复发发作时均发生呼吸道感染。4 例患者（28.6%）对治疗有抵抗性，分别与牙脓肿（1 例）、感染性休克（1 例）和肺炎支原体感染（2 例）有关。所有 4 例患者的 CRP 值均明显升高，中位数为 335mg/L。
大多数与感染相关的发作发生在冬季（77.8%）。对于特发性 TTP 患者，在常规治疗无效的情况下，应认真考虑感染作为其初始和/或复发发作的额外致病因素，特别是当 CRP 明显升高时。
