Glucose-6-phosphate dehydrogenase

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic disorder that affects 400 million people worldwide, mostly men with a high prevalence in persons of African, Asian, and Mediterranean descent (Nagalla, et al., 2017). “The distribution across Asia is heterogeneous. One of the G6PD variants can be found in 20% of males in Thailand. It is common in southern China and rare in other parts of China, while prevalence varies in India” (Wahed & Dasgupta, 2015). This condition prematurely breaks down red blood cells causing acute and chronic hemolysis, anemia and jaundice. Patients with this disorder can experience episodes of hemolysis after been exposed to infections. Even ingesting fava beans can potentially cause hemolysis. “Many individuals with G6PD deficiency are asymptomatic and often do not need treatment. However, they need to be educated to avoid chemicals and drugs that can cause oxidant stress (Nagalla, 2017). Some medications they should avoid are antimalarial primaquine, NSAIDs, aspirin, quinine, nitrofurans, sulfonamides and rasburicase.
Rasburicase is a DNA recombinant urate oxidase that is used in the treatment and prevention of hyperuricemia in patients with leukemia, lymphoma, and solid tumor malignancies who are receiving chemotherapy with the expected result of tumor lysis and elevation of plasma uric acid (Wilson & Bern, 2014). It has also been used to treat acute gout in patients who responded poorly to allopurinol who developed severe urticarial or angioedema (Wahed & Dasgupta, 2015). Rasburicase converts uric acid into allantoin, carbon dioxide, and hydrogen peroxide, which is then excreted by the kidneys. Rasburicase lowers serum uric acid quickly and has few side effects. The black box warning states do not administer to patients with G6PD deficiency. Therefore, patients should be screened prior to administration to determine if they have G6PD deficiency. Per the Clinical Pharmacogenetics Implementation Consortium (CPIC), universal neonatal screening programs for G6PD deficiency via the use of semiquantitative fluorescent spot test or quantitative enzyme activity assay have been instituted or proposed in areas with a high incidence of G6PD deficiency such as Asia, Europe, Africa, and the Middle East (Relling et al., 2014). Rasburicase is also contraindicated in patients who are pregnant or lactating.