Abdulsalam Abbas Hashim, Al-Rahal Nidhal Karim, Ghiath Yusra
AL-Mamoon University College and Baghdad Teaching Hospital, Baghdad, Iraq.
The National Center of Hematology, Mustansiriyah University, Baghdad, Iraq.
Indian J Hematol Blood Transfus. 2021 Jan;37(1):96-100. doi: 10.1007/s12288-020-01306-8. Epub 2020 Jun 10.
Inherited bleeding disorders can lead to lifelong bleeding; they are mainly caused by quantitative or qualitative defect of coagulation factors, von Willebrand factor (VWF) or platelets. No published data are available about the different types of inherited bleeding disorders in Iraq.
To describe types, severity and presentation of inherited bleeding disorders in pediatric patients in the major referral center in Iraq.
This is a cohort prospective descriptive study conducted at the National Center of Hematology, a referral center for bleeding disorders in Baghdad-Iraq, from January 2015 to December 2019. One hundred ninety-one patients, aged 1 day to 14 years, with suspected inherited bleeding disorder are included in this study. Each patient was interviewed, accompanied by a chaperone, mostly parent(s), with revision of personal and familial bleeding history, conducting a brief medical examination, and withdrawing blood for complete blood count, peripheral blood film, bleeding time, PT and APTT. Further investigations including mixing studies, lupus anticoagulant, clotting factor activity, VWF:antigen (VWF:Ag), VWF: Ristocetin cofactor (VWF:RiCof) and platelet function tests using light transmission aggregometry were performed only if indicated.
The mean ± 1SD of age of patients is 5.3 ± 3 years, with a male:female ratio of 1.3:1. Family history of a similar bleeding disorder is recorded in 44.9% patients ( < 0.05). Consanguineous marriage was observed in 70.8% of the families ( < 0.001). The most prevalent inherited bleeding disorder is von Willebrand disease (VWD) (43.9%), of which type 3 is the most common (86.9%). Thrombasthenia is the second most prevalent (39.8%) inherited bleeding disorder; of these, the majority have Glanzmann's thrombasthenia (82.9%). Hemophilia A is found in 9.4% of patients.
Type 3 VWD, Glanzmann's thrombasthenia and hemophilia A are the most common inherited bleeding disorders in the central part of Iraq, collectively they constitute >86% of patients. Consanguineous marriage should be discouraged in our society to decrease hereditary bleeding disorders. Also, there is a need to increase awareness and knowledge of bleeding disorders to improve early identification, mitigate the risk of further bleeding and prevent complications.
遗传性出血性疾病可导致终身出血；它们主要由凝血因子、血管性血友病因子（VWF）或血小板的数量或质量缺陷引起。关于伊拉克遗传性出血性疾病的不同类型，尚无公开数据。
描述伊拉克主要转诊中心儿科患者遗传性出血性疾病的类型、严重程度和表现。
这是一项队列前瞻性描述性研究，于2015年1月至2019年12月在伊拉克巴格达的国家血液学中心进行，该中心是出血性疾病的转诊中心。本研究纳入了191例年龄在1天至14岁之间、疑似患有遗传性出血性疾病的患者。每位患者均在一名陪同人员（大多为父母）的陪同下接受访谈，回顾个人和家族出血史，进行简要体格检查，并抽取血液进行全血细胞计数、外周血涂片、出血时间、凝血酶原时间（PT）和活化部分凝血活酶时间（APTT）检查。仅在有指征时才进行进一步检查，包括混合试验、狼疮抗凝物、凝血因子活性、VWF：抗原（VWF：Ag）、VWF：瑞斯托霉素辅因子（VWF：RiCof）以及使用光透射聚集法进行血小板功能测试。
患者的平均年龄±1标准差为5.3±3岁，男女比例为1.3：1。44.9%的患者有类似出血性疾病的家族史（P<0.05）。70.8%的家庭存在近亲结婚情况（P<0.001）。最常见的遗传性出血性疾病是血管性血友病（VWD）（43.9%），其中3型最为常见（86.9%）。血小板无力症是第二常见的（39.8%）遗传性出血性疾病；其中，大多数为Glanzmann血小板无力症（82.9%）。9.4%的患者患有甲型血友病。
3型VWD、Glanzmann血小板无力症和甲型血友病是伊拉克中部最常见的遗传性出血性疾病，它们总共占患者的比例超过86%。应在我们的社会中劝阻近亲结婚，以减少遗传性出血性疾病。此外，有必要提高对出血性疾病的认识和了解，以改善早期识别、降低进一步出血的风险并预防并发症。
