Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.
Am J Hematol. 2024 Nov;99(11):2063-2074. doi: 10.1002/ajh.27458. Epub 2024 Aug 13.
Prior studies have suggested that immune thrombotic thrombocytopenic purpura (iTTP) may display seasonal variation; however, methodologic limitations and sample sizes have diminished the ability to perform a rigorous assessment. This 5-year retrospective study assessed the epidemiology of iTTP and determined whether it displays a seasonal pattern. Patients with both initial and relapsed iTTP (defined as a disintegrin and metalloprotease with thrombospondin type motifs 13 activity <10%) from 24 tertiary centers in Australia, Canada, France, Greece, Italy, Spain, and the US were included. Seasons were defined as: Northern Hemisphere-winter (December-February); spring (March-May); summer (June-August); autumn (September-November) and Southern Hemisphere-winter (June-August); spring (September-November); summer (December-February); autumn (March-May). Additional outcomes included the mean temperature in months with and without an iTTP episode at each site. A total of 583 patients experienced 719 iTTP episodes. The observed proportion of iTTP episodes during the winter was significantly greater than expected if equally distributed across seasons (28.5%, 205/719, 25.3%-31.9%; p = .03). Distance from the equator and mean temperature deviation both positively correlated with the proportion of iTTP episodes during winter. Acute iTTP episodes were associated with the winter season and colder temperatures, with a second peak during summer. Occurrence during winter was most pronounced at sites further from the equator and/or with greater annual temperature deviations. Understanding the etiologies underlying seasonal patterns of disease may assist in discovery and development of future preventative therapies and inform models for resource utilization.
先前的研究表明,免疫性血栓性血小板减少性紫癜(iTTP)可能存在季节性变化;然而,方法学上的限制和样本量的减少降低了进行严格评估的能力。这项为期 5 年的回顾性研究评估了 iTTP 的流行病学,并确定其是否存在季节性模式。来自澳大利亚、加拿大、法国、希腊、意大利、西班牙和美国的 24 个三级中心的初次和复发性 iTTP(定义为去整合素和金属蛋白酶与血小板反应蛋白 13 活性<10%)患者均纳入本研究。季节定义为:北半球冬季(12 月至 2 月);春季(3 月至 5 月);夏季(6 月至 8 月);秋季(9 月至 11 月)和南半球冬季(6 月至 8 月);春季(9 月至 11 月);夏季(12 月至 2 月);秋季(3 月至 5 月)。其他结果包括每个地点在有 iTTP 发作和无 iTTP 发作的月份中的平均温度。共有 583 例患者经历了 719 次 iTTP 发作。如果在各个季节均匀分布,观察到的 iTTP 发作比例在冬季明显高于预期(28.5%,205/719,25.3%-31.9%;p=0.03)。距赤道的距离和平均温度偏差均与冬季 iTTP 发作比例呈正相关。急性 iTTP 发作与冬季和较低温度相关,夏季有第二个高峰。距离赤道越远和/或年温度偏差越大,冬季发作的发生率越高。理解疾病季节性模式背后的病因可能有助于发现和开发未来的预防疗法,并为资源利用模型提供信息。