Britannica Sources used in this answer

How does glucose-6-phosphate dehydrogenase deficiency lead to hemolytic anemia?

Created from Britannica articles using AI. AI can make mistakes, so verify using Britannica articles.

blood disease Peripheral blood smear revealing histopathologic features indicative of a blast crisis in a case of chronic myelogenous leukemia (CML). Blast crisis occurs when one-fifth to one-third of the cells in the blood or bone marrow are immature blood cells, or blast cells. This phase of CML is characterized by fever, weakness, and an enlarged spleen.

Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency leads to hemolytic anemia by impairing the red blood cell’s ability to protect itself from oxidative damage.1

Normally, the enzyme G-6-PD functions within the pentose phosphate pathway to help generate substances that prevent the oxidative destruction of the cell membrane. When G-6-PD activity is markedly reduced, these protective mechanisms are compromised.12

While affected cells may remain relatively stable under normal conditions, exposure to certain oxidant drugs—such as sulfonamides or specific antimalarial compounds like primaquine—triggers the oxidative destruction of the cell. This process causes the red blood cells to break down prematurely, a condition known as hemolysis. If the rate of this destruction exceeds the body's capacity to produce new red cells, hemolytic anemia develops. Because the gene for this enzyme is sex-linked, the condition is most fully expressed in males.12