Ashfaq Javeria, Tariq Faryal, Ahmed Rehana, Thakur Warkha, Abid Madiha, Borhany Munira
Clinical Haematology, National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi, PAK.
Haematology, National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi, PAK.
Cureus. 2022 Jun 16;14(6):e26008. doi: 10.7759/cureus.26008. eCollection 2022 Jun.
To determine the frequency of specific and non-specific inhibitors in haemophilia A patients.
This is a cross-sectional study.
A total of 150 male haemophilia A patients were included in this cross-sectional study at the National Institute of Blood Diseases and Bone Marrow Transplant (NIBD), Karachi, Pakistan, from September 2019 to January 2022.
Among 150 patients included in this study, 23 (15.3%) had an inhibitor and 127 (84.6%) did not. All patients had specific inhibitors against Factor VIII (FVIII). Non-specific inhibitors were not identified in our population. Among the patients in the inhibitor group, there were 13 (56.5%) in the severe (<1%) category. There were 10 (43.5%) patients in the moderate (1-5%) category. There were no patients in the mild category. The median inhibitor level was 15.4 Bethesda unit (BU).
The development of inhibitors has not been identified as a major problem in our population. However, it is noteworthy that only 15.3% of patients with haemophilia A developed inhibitors in this data set. They were essentially treated with plasma and its products.
确定甲型血友病患者中特异性和非特异性抑制剂的发生率。
这是一项横断面研究。
2019年9月至2022年1月期间，在巴基斯坦卡拉奇国家血液疾病和骨髓移植研究所（NIBD），共有150名男性甲型血友病患者纳入了这项横断面研究。
在本研究纳入的150名患者中，23名（15.3%）有抑制剂，127名（84.6%）没有。所有患者均有针对凝血因子VIII（FVIII）的特异性抑制剂。在我们的研究人群中未发现非特异性抑制剂。在抑制剂组患者中，重度（<1%）类别中有13名（56.5%）。中度（1-5%）类别中有10名（43.5%）患者。轻度类别中没有患者。抑制剂水平中位数为15.4贝塞斯达单位（BU）。
在我们的研究人群中，抑制剂的产生尚未被确定为一个主要问题。然而，值得注意的是，在该数据集中，只有15.3%的甲型血友病患者产生了抑制剂。他们主要接受血浆及其制品治疗。
