Hematological, Biochemical Properties, and Clinical Correlates of Hemoglobin S Variant Disorder: A New Insight Into Sickle Cell Trait

Abstract

Background The sickle cell trait (SCT) disorder possesses a clinical heterogeneity ranging from a symptomless condition to sudden death. This study aimed to develop a diagnostic approach that helps the characterization and identification of SCT from normal subjects and sickle cell disease (SCD) patients, and to assess its severity. Methods Sixty controls, 24 SCD patients and 31 SCT subjects were assessed clinically, radiologically and by laboratory investigations. Results Of the SCT subjects, 12.8% were symptomatic (3.2% anemic, 6.4% hemolytic crisis, and 3.2% painful crises). Anemia was normocytic in 66.6%, and normochromic and polychromatic in 33.4%. Significantly lower red blood cells (RBCs), hemoglobin (Hb), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), hematocrit (Hct), Shine and Lal index (SL), and hemoglobin A (Hb A), and higher mean corpuscular hemoglobin concentration (MCHC), red cell distribution width (RDW), Ricerca index (RI), and Huber-Herklotz index (HH) were found in SCT subjects compared with the controls. Hb A and hemoglobin S (Hb S) were excellent in discriminating SCT from SCD (cut-off for SCT > 50% and < 40%) followed by Hct, MCHC, Hb, Green and King index (GK), and England and Fraser index (EF) (cut-off for SCT > 33%, > 32, > 11, < 71, and < 10, respectively). Radiologically normal findings were detected in 87% of SCT subjects; they had nearly normal liver and renal function tests (except one case each). A schematic diagnostic paradigm for SCT was proposed. Conclusion This study allowed understanding of SCT in various aspects, i.e., clinical, hematological, biochemical and radiological. Thus, it could help prevention of the Hb S variant disorder and proper management of carriers. This might be applied in pre-marital screening, particularly in those with family history of Hb S disorder.

Sickle cell anemia (SCA) and the impact of G6PD activity on the Hematological parameters

“…In the current study, we found significantly lower Hb, PCV, and MCHC and higher reticulocytes in SCA patients than in SCT patients. Our findings were in agreement with other studies [19]. Also, we found higher MCHC in SCT patients compared to SCD patients, while MCHC was low in SCD patients.…”

“…Also, non-significant differences were obtained in age, gender, leukocytes, platelets, MCV, MCH, MCHC, and G6PD between SCD patients and controls (P ˃ 0.05). Similar results were obtained in other studies by comparing SCA patients with controls [19]. Khaled et al, (2022) found significantly lower levels of Hb, PCV, RBC, MCV, and MCH and higher levels of reticulocytes in SCD patients than controls, as well as non-significant differences in age, gender, WBC, platelets, and MCHC [19].…”

Genome-Wide Analysis of Exertional Rhabdomyolysis in Sickle Cell Trait Positive African Americans

“…The percentage of HbS, which ranges from 25% to 45% [50], may affect the hematological and clinical characteristics of SCT, including ERM [51]. However, the Kennedy et al study suggested that HbS levels in SCT were very unlikely to have any clinical significance [52].…”