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与胎儿血红蛋白水平升高相关的炎性细胞因子可减少镰状细胞病患者的血管闭塞性危象发作次数。

Inflammatory Cytokines in Association With High Fetal Hemoglobin Level Reduce the Episodes of Vaso-Occlusive Crisis in Sickle Cell Patients.

作者信息

Dixit Sujata, Das Arundhuti, Samal Priyanka, Sandeepta Sonali, Panigrahi Swati Sudeshna, Jain Hitesh Kumar, Biswas Subhojeet, Suar Mrutyunjay, Pati Sanghamitra, Ranjit Manoranjan, Bal Madhusmita

机构信息

Department of Immunology, Indian Council of Medical Research (ICMR)-Regional Medical Research Centre, Bhubaneswar 751023, Odisha, India.

School of Biotechnology, Kalinga Institute of Industrial Technology (KIIT) University, Bhubaneswar 751024, Odisha, India.

出版信息

J Hematol. 2025 Jun;14(3):124-132. doi: 10.14740/jh1353. Epub 2025 Apr 14.

Abstract

BACKGROUND

Vaso-occlusive crisis (VOC), a common clinical manifestation of sickle cell disease (SCD), is mediated by a series of inflammatory responses. Conversely, a high fetal hemoglobin (Hb F) level is a known factor that reduces the severity of clinical presentations associated with SCD. Since the association between cytokine profiles and high Hb F levels in SCD is not well studied, this study aims to investigate the interaction between cytokines and Hb F levels in SCD patients from Odisha, India.

METHODS

A total of 276 patients with SCD were recruited for the study, including 180 in steady state and 90 in crisis state. Additionally, 117 individuals with sickle cell trait (SCT) and 128 healthy controls were included for comparison.

RESULTS

The study revealed that the total white blood cell count and interleukin (IL)-6 levels were significantly higher in crisis state SCD patients, while the red cell distribution width, IL-10, and Hb F level were significantly higher in steady state SCD patients. Most importantly, the logistic regression analysis showed that the interaction of Hb F with the cytokines IL-10, IL-1β, and IL-17 provided three times greater protection from crisis in SCD patients.

CONCLUSION

The significance of these preliminary findings has been discussed in terms of prognostic markers and supplements to increase the efficacy of hydroxyurea used to enhance Hb F production.

摘要

背景

血管闭塞性危机(VOC)是镰状细胞病(SCD)的常见临床表现,由一系列炎症反应介导。相反,高胎儿血红蛋白(Hb F)水平是降低与SCD相关临床表现严重程度的已知因素。由于SCD中细胞因子谱与高Hb F水平之间的关联尚未得到充分研究,本研究旨在调查印度奥里萨邦SCD患者中细胞因子与Hb F水平之间的相互作用。

方法

本研究共招募了276例SCD患者,包括180例病情稳定患者和90例处于危机状态的患者。此外,纳入117例具有镰状细胞特征(SCT)的个体和128例健康对照进行比较。

结果

研究显示,处于危机状态的SCD患者的白细胞总数和白细胞介素(IL)-6水平显著更高,而病情稳定的SCD患者的红细胞分布宽度、IL-10和Hb F水平显著更高。最重要的是,逻辑回归分析表明,Hb F与细胞因子IL-10、IL-1β和IL-17的相互作用为SCD患者提供了三倍的保护,使其免受危机影响。

结论

已根据预后标志物以及增加用于提高Hb F生成的羟基脲疗效的补充剂对这些初步发现的意义进行了讨论。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb86/12151094/4bcf16281ee1/jh-14-03-124-g001.jpg

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