Impact of ABO blood group antigens on residual factor VIII levels and risk of inhibitor development in hemophilia A.

IF 2.3 Q2 HEMATOLOGY
Debadrita Ray, Narender Kumar, Chander Hans, Anita Kler, Richa Jain, Deepak Bansal, Amita Trehan, Arihant Jain, Pankaj Malhotra, Jasmina Ahluwalia
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Abstract

Background: The clinical phenotype of hemophilia A (HA) does not always correlate with severity. Similarly, the presence of inhibitors does not necessarily increase the risk of bleeding. This paradox between clinical and laboratory findings may be partially attributed to non-modifiable factors, such as blood group, which is known to influence FVIII levels in healthy individuals. Our aim was to assess the effect of ABO blood group antigens on FVIII levels across the severity spectrum of HA and risk of inhibitor development.

Methods: Data of consecutive patients with HA who visited the coagulation unit of a northern Indian tertiary care hospital between 2010‒2021 were reviewed. Patients with missing blood group data, transfusion histories, or baseline FVIII levels were excluded.

Results: Mild, moderate, and severe HA was present in 41 (6.9%), 72 (12.2%), and 479 (80.9%) patients, respectively. There were no differences in the FVIII levels among the various blood groups across the HA severity spectrum. Inhibitors were administered to 35 patients (5.9%). In the multivariate analysis, blood group A was an independent risk factor for the development of inhibitors (adjusted odds ratio 2.70, P=0.04) after adjusting for age at onset of bleeding, FVIII transfusion, age at first FVIII transfusion, and severity of HA.

Conclusion: Unlike what is observed in healthy individuals, blood group did not influence residual FVIII levels across the severity spectrum of HA. Patients in group A had a higher risk of developing inhibitors.

Abstract Image

ABO血型抗原对血友病A患者残留因子VIII水平及抑制剂发展风险的影响
背景:血友病A (HA)的临床表型并不总是与严重程度相关。同样,抑制剂的存在并不一定会增加出血的风险。临床和实验室结果之间的矛盾可能部分归因于不可改变的因素,如血型,已知会影响健康个体的FVIII水平。我们的目的是评估ABO血型抗原对HA严重程度范围内FVIII水平和抑制剂发展风险的影响。方法:回顾2010-2021年间在印度北部三级医院凝血病房连续就诊的HA患者的资料。排除了血型数据、输血史或基线FVIII水平缺失的患者。结果:轻度、中度和重度HA患者分别为41例(6.9%)、72例(12.2%)和479例(80.9%)。在HA严重程度的不同血型中,FVIII水平没有差异。35例患者(5.9%)使用抑制剂。在多因素分析中,在调整出血年龄、FVIII输血年龄、首次FVIII输血年龄和HA严重程度后,A血型是抑制剂发生的独立危险因素(校正优势比2.70,P=0.04)。结论:不同于在健康个体中观察到的情况,血型不影响HA严重程度谱上的残留FVIII水平。A组患者出现抑制剂的风险更高。
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来源期刊
Blood Research
Blood Research HEMATOLOGY-
CiteScore
3.70
自引率
0.00%
发文量
64
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