Background: Glucose-6-Phosphate Dehydrogenase (G6PD) insufficiency is a hereditary condition characterised by a lack of or inability to produce G6PD, an enzyme necessary for red blood cell activity. G6PD gene mutations are inherited in a sex-linked recessive fashion. Males and either homozygous or heterozygous females with an inactive X chromosome are so impacted (lyonized females). Autoimmune hemolytic anaemia (AIHA) is a condition marked by the early destruction of red blood cells (RBC) by autologous RBC autoantibodies, as shown by a positive direct antiglobulin (Coombs) test (DAT). Based on the lack or presence of an underlying illness, AIHA is divided into primary or idiopathic and secondary forms. In Nigeria, children with AIHA are uncommon, while secondary forms are frequent among teens. Chronic granulomatous disease (CGD) is a rare hereditary immunological condition marked by neutrophil dysfunction and a lack of G6PD in both neutrophils and red blood cells. CGD is inherited in a sex-linked recessive and autosomal recessive form. As a result, CGD with G6PD might arise in a female with an X chromosomal defect, where the presence of an immunological condition and G6PD deficiency could exacerbate hemolysis.
The goal is to raise awareness about the possibility of a rare chronic granulomatous illness.
Case: A case of acute hemolytic anaemia in an adolescent female with G6PD deficiency, a positive direct Coombs test, and apparent neutrophil dysfunction.
A 14-year-old female with systemic inflammatory response syndrome, significant neutrophilia but minor toxic granulation, steroid-responsive hemolytic anaemia, and a positive direct Coombs test is described.
Conclusion: In an adolescent girl, C6PD deficiency and AIHA may co-exist as part of CGD, but a clear diagnosis of CGD is necessary.
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