Comorbid glucose-6-phosphate dehydrogenase (G6PD) deficiency exacerbates the clinical course of patients with sickle cell disease (SCD), according to findings published in Hematology. Like SCD, G6PD enzyme deficiency impacts erythrocytes, causing premature hemolysis which promotes anemia.

Researchers analyzed the data obtained from 122 patients with SCD treated at 5 health centers in Kisangani City in the Democratic Republic of the Congo (DRC) from September 28, 2019, to March 4, 2021. They compared the patients with SCD to 211 healthy controls who donated blood at a provincial blood transfusion center.

During the clinical examination, the patients with SCD reported various SCD-related complications, including the number of transfusions within the past 6 months, the number of SCD- related hospitalizations in the past year, and the frequency of painful attacks or anemia requiring transfusion in the past year.

The researchers obtained the hemoglobin phenotype, hemoglobin levels, reticulocyte levels, and G6PD enzyme activity from laboratory blood tests.

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G6PD deficiency presented in 34.2% of all trial participants and more commonly occurred in males (P =.038). The researchers did not observe any significant difference in the prevalence of G6PD deficiency between the SCD and control groups (35.2% vs 33.6%, P =.767). Prevalence of G6PD deficiency also did not differ significantly among hemoglobin phenotypes (P =.214).

Although it did not attain statistical significance, non-G6PD deficient patients with SCD exhibited higher hemoglobin levels than those with both SCD and G6PD deficiency (6000±1687 g/dL vs 5605±2172 g/dL, P =.964).

In contrast, the clinical presentations of G6PD vs non-G6PD deficient patients with SCD did differ significantly. Patients with both SCD and G6PD deficiency experienced more transfusions (0.64±0.897 vs 0.24±0.486; P =.004), SCD-related hospitalizations (1.42 ± 1.451 vs 0.76±1.112; P =.007), major vaso-occlusive crises (1.37±1.092 vs 0.85±1.014; P =.005), and anemia requiring transfusion (0.74±0.902 vs 0.38±0.739; P =.007).

“The G-6-PD deficiency appears to worsen the clinical features of SCD,” the authors concluded. “Therefore, a systematic screening for G6PD deficiency in all SCD patients in the DRC is essential and should be considered to prevent the occurrence of iatrogenic hemolytic accidents.”

Reference

Kambale-Kombi P, Marini Djang’eing’a R, Alworong’a Opara JP, et al. Does glucose-6-phosphate dehydrogenase deficiency worsen the clinical features of sickle cell disease? A multi-hospital-based cross-sectional study. Hematology. 2022;27(1):590-595. doi:10.1080/16078454.2022.2074715