Innate immune response to bacterial infections in hospitalized glucose-6-phosphate dehydrogenase-deficient patients;...

Inherited hematological disorders, affecting immune cell quantity and function, compromise immune system efficiency. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common X-linked inherited enzymopathy and has been implicated in compromised immune cell function and host defense, and in elevating infection vulnerability. This study explores the impact of G6PD deficiency on immune cellular response and infection-related mortality.

Adults with G6PD deficiency and bacterial infections (2017-2021) were analyzed. Demographics, comorbidities, laboratory parameters, and microbiological data were assessed. Cox proportional hazards regression analyzed the association between white blood cell (WBC) count and 28-day mortality. The data analysis was conducted using the R software statistical programming language.

The investigation included 334 bacterial infection episodes representing 202 unique patients who fulfilled the inclusion criteria. A 19.2 % 28-day mortality rate was observed. Males, those aged ≥60, and patients with comorbidities had higher mortality. Anemia, high globulin, and elevated liver enzymes were associated with mortality. The majority of patients demonstrated reduced WBC counts (<9.8 × 10⁹/L) during infection, significantly contributing to increased mortality risk (HR: 1.95, p = 0.008). Males exhibited a more pronounced impact. This study reveals heightened mortality risk in G6PD-deficient patients with diminished WBC counts during infections. Elevated CRP, globulins, and liver enzymes indicated severe infections, contrasting the unexpected non-response in WBC counts.

Vigilance in treating infections in G6PD-deficient patients, especially in males, is crucial for favorable outcomes. Diagnostic reliance on altered WBC counts in this population may misguide clinical decisions. G6PD deficiency poses a unique challenge in infection management, emphasizing the need for tailored interventions and exploration of alternative biomarkers for assessing severity and predicting mortality. Further studies and larger cohorts are essential for a comprehensive understanding and improved clinical practices.