Glucose-6-Phosphate Dehydrogenase Deficiency-Associated Hemolytic Anemia and Methemoglobinemia in a Patient Treated With Hydroxychloroquine in the Era of COVID-19.

Nicole Laslett,Julianne Hibbs,Vlasta Zemba-Palko,Arezoo Ghaneie,Max Hallett

doi:10.7759/cureus.15232

Abstract

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymatic disorder of red blood cells worldwide. The severity of hemolytic anemia varies among individuals with G6PD deficiency, depending on the genetic variant in the G6PD gene; this makes the diagnosis of the condition more challenging in some cases. In this report, we present a case of severe hemolytic anemia and methemoglobinemia in a patient with G6PD deficiency who had been exposed to hydroxychloroquine prescribed for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. To the best of our knowledge and based on a literature search, this is one of the first case reports in the literature about hemolytic crisis and methemoglobinemia in a patient with critical illness due to severe coronavirus disease 2019 (COVID-19) who was exposed to hydroxychloroquine. It is critical for physicians and caregivers to recognize the effects of oxidative stressors such as hydroxychloroquine, particularly in this era of the COVID-19 pandemic and in regions with a high prevalence of G6PD deficiency, for the appropriate management of this unique subset of patients.

Highlights

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited disorder caused by a genetic defect in the red blood cell enzyme G6PD, affecting around 400 million people worldwide [1]
Patients with G6PD deficiency may be more vulnerable to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection as G6PD-deficient lung cells infected with human coronavirus 229E result in increased viral production and replication compared with normal cells [4]
The use of hydroxychloroquine may trigger severe acute hemolytic crisis in G6PD-deficient patients, and there is scarce data on this disease in the literature, and it may pose serious challenges especially during the ongoing COVID-19 pandemic, when hydroxychloroquine is routinely prescribed for affected patients

Summary

Introduction

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited disorder caused by a genetic defect in the red blood cell enzyme G6PD, affecting around 400 million people worldwide [1]. Laboratory analysis revealed acute kidney injury, electrolyte abnormalities, high anion gap metabolic acidosis and lactic acidosis, and elevated inflammatory markers with normal blood counts He was started on antibiotics for presumed community-acquired pneumonia and hydroxychloroquine (800 mg on hospital day one, 400 mg on hospital day two) for severe COVID-19 infection. G6PD level was found to be 19.8 U/g Hgb (7.0-20.5 U/g Hgb) on hospital day seven This was suggestive of methemoglobinemia and severe hemolytic crisis due to underlying G6PD deficiency in the setting of hydroxychloroquine administration and severe COVID-19 illness. G6PD: glucose-6-phosphate dehydrogenase; MetHgb: methemoglobin; Bili: bilirubin; Hapto: haptoglobin; Hgb: hemoglobin; LDH: lactate dehydrogenase; Vit C: vitamin C

Discussion

Conclusions

Disclosures

Glader B

Findings

Beutler E