Certified Pediatric Hematology Oncology Nurse (CPHON) Practice Exam

In the context of G6PD deficiency, the most significant and common occurrence is hemolysis of red blood cells. G6PD (Glucose-6-Phosphate Dehydrogenase) is an enzyme that plays a vital role in the pentose phosphate pathway, which generates NADPH. NADPH is essential for maintaining adequate levels of glutathione, an important antioxidant that protects red blood cells from oxidative damage.

When a person has G6PD deficiency, there is insufficient NADPH produced, leading to a decrease in glutathione levels. This deficiency makes red blood cells more susceptible to oxidative stress, which can be triggered by various factors such as infections, certain medications, and foods (like fava beans). The result of this oxidative stress is the premature destruction of red blood cells, a process known as hemolysis. During hemolysis, red blood cells rupture, leading to anemia and a range of associated symptoms.

While decreased white blood cell count, increased risk of infections, and growth retardation may also occur in certain conditions affecting the immune or overall health, they are not the primary concern associated with G6PD deficiency. The hallmark feature of this deficiency is indeed the hemolytic anemia due to the lack of protection from