Publication:

Association of hemolysis markers and cortisol level with different severity of sickle cell disease among Sudanese patients

Background Sickle cell anemia (SCA) is an inherited disorder of hemoglobin. Several biomarkers have been identified, which is essential in the different clinical presentations of the disease. This study aimed to determine the association between hemolysis markers and cortisol level with varying severity groups of Sudanese patients with SCA.

Methods This descriptive cross-sectional study included 100 patients with sickle cell disease between February 2016 and April 2017. According to Hedo et al scoring, medical history was obtained to conduct disease severity. A total of 3 ml of venous blood was collected from each patient. A complete hemogram was performed using an automated hematology analyzer (Sysmix®-KX-21N). Bilirubin and lactate dehydrogenase (LDH) were estimated using a spectrophotometer, while cortisol was measured using the Elecsys® system 2010 E170. The reticulocyte count was performed manually. Data were analyzed using statistical package for the social sciences (SPSS) version 21 computer software program.

Results Disease severity was variable and was categorized into; eighteen (18%) patients had mild symptoms, while 70 (70%) patients had moderate disease, and 12 (12%) patients had severe disease. The analysis of variance (ANOVA) test showed that hemoglobin, reticulocyte count, LDH, and direct bilirubin were positively correlated with disease severity, p value: 0.001, 0.04, 0.00, and 0.02, respectively. While indirect bilirubin, total bilirubin, and cortisol did not correlate with disease severity, the p value was (0.248, 0.083, and 0.868, respectively).

Conclusions This study confirmed that the hemolysis markers (Hb, reticulocyte count, direct bilirubin and LDH) were positively associated with disease severity. In contrast, indirect bilirubin, total bilirubin, and cortisol levels were not associated with the disease severity.