Department of Internal Medicine.
Department of Medical Biology, Istanbul Faculty of Medicine, Istanbul University.
Blood Coagul Fibrinolysis. 2024 Sep 1;35(6):307-315. doi: 10.1097/MBC.0000000000001318. Epub 2024 Jul 24.
Thrombotic thrombocytopenic purpura (TTP) is one of the rare group disorders classified as thrombotic microangiopathies (TMAs). Approximately 90% of TTP developed immune-mediation by the formation of antibodies against the enzyme ADAMTS-13. The exact cause is unknown. To establish an association between human leukocyte antigen (HLA) and autoimmune basis, as susceptibility or protection against the disease, we contributed a study aiming to evaluate the role of HLA in immune-mediated TTP (iTTP). Considering epidemiological factors such as age, sex, ethnicity, and geographical origins, we contributed the study in our country, Turkey, which consist of a very heterogeneous population. Patients' data collection was retrospectively from electronic database on two University hospitals having big therapeutic apheresis service. Control arm was healthy people registered as stem cell donors matched in terms of age and sex. The frequency of HLA-DRB1 and HLA-DQB1 alleles between acquired TTP and the control group was compared using the chi-square method. Yates correction and logistic regression were performed on these results. A total of 75 iTTP patients and 150 healthy individuals enrolled to the study. HLA-DRB1∗11, HLA-DQB1∗03, HLA-DRB1∗11:01, HLA-DRB1∗14:01, HLA-DRB1∗13:05, HLA-DRB1∗11 + HLA-DQB1∗03 allele pair and HLA-DRB1∗15 + HLA- DQB1∗06 were proved to be susceptibility allele pairs for iTTP. HLA-DRB1∗15, HLA-DRB1∗01:01, HLA-DRB1∗07:01, HLA-DRB1∗13:01, HLA-DRB1∗14:54, HLA-DQB1∗05:01, HLA-DQB1∗02:02 and HLA-DRB1∗07 + HLA-DQB1∗02 allele pair were found to be protective against iTTP. Our findings support an association with iTTP across very heterogenous populations in Turkey.
血栓性血小板减少性紫癜（TTP）是一种罕见的血栓性微血管病（TMA）分类疾病。大约 90%的 TTP 是由针对 ADAMTS-13 酶的抗体形成引起的免疫介导。确切的原因尚不清楚。为了在人类白细胞抗原（HLA）和自身免疫基础之间建立关联，作为对疾病的易感性或保护，我们进行了一项研究，旨在评估 HLA 在免疫介导的 TTP（iTTP）中的作用。考虑到年龄、性别、种族和地理起源等流行病学因素，我们在土耳其进行了这项研究，土耳其是一个人口非常多样化的国家。患者数据是从两家拥有大型治疗性血浆去除服务的大学医院的电子数据库中回顾性收集的。对照组是作为年龄和性别匹配的干细胞供体登记的健康人。使用卡方检验比较获得性 TTP 患者和对照组之间 HLA-DRB1 和 HLA-DQB1 等位基因的频率。对这些结果进行了 Yates 校正和逻辑回归。共有 75 例 iTTP 患者和 150 名健康个体纳入研究。HLA-DRB1∗11、HLA-DQB1∗03、HLA-DRB1∗11:01、HLA-DRB1∗14:01、HLA-DRB1∗13:05、HLA-DRB1∗11 + HLA-DQB1∗03 等位基因对和 HLA-DRB1∗15 + HLA-DQB1∗06 被证明是 iTTP 的易感等位基因对。HLA-DRB1∗15、HLA-DRB1∗01:01、HLA-DRB1∗07:01、HLA-DRB1∗13:01、HLA-DRB1∗14:54、HLA-DQB1∗05:01、HLA-DQB1∗02:02 和 HLA-DRB1∗07 + HLA-DQB1∗02 等位基因对被发现对 iTTP 具有保护作用。我们的研究结果支持在土耳其非常多样化的人群中与 iTTP 存在关联。
