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

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.

Keywords: Cytokine; Fetal hemoglobin; IL-6; Sickle cell disease; VOC.

Figure 1

Association between cytokines and sickle cell disease patients in steady and crisis state, and healthy controls.