Review Question - QID 210922

This patient who experienced jaundice, back pain, and anemia after starting primaquine for latent malaria most likely has G6PD deficiency. In this disease, small round inclusions of denatured hemoglobin known as Heinz bodies can be seen within erythrocytes on peripheral blood smear.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked genetic disorder that can result in intrinsic hemolytic anemia. This enzyme is normally involved in the generation of nicotinamide dinucleotide phosphate (NADPH), which protects red blood cells against oxidative stress. In the absence of this protective mechanism, oxidative triggers will lead to red blood cell lysis. Known triggers include primaquine, dapsone, sulfa drugs, infections, and fava bean ingestion. Since red blood cells in this disorder are not able to resist stress, hemoglobin will denature and become small round inclusions known as Heinz bodies.

Incorrect Answers:
Answer 1: Helmet-shaped red blood cells known as schistocytes are seen in microangiopathic disorders; however, they would not be seen in G6PD deficiency.

Answer 3: Small round inclusions of nuclear remnants known as Howell-Jolly bodies are seen in asplenic patients; however, they would not be seen in G6PD deficiency.

Answer 4: Target-shaped red blood cells known as codocytes are seen in a wide variety of anemias such as thalassemia; however, they would not be seen in G6PD deficiency.

Answer 5: Teardrop shaped red blood cells known as dacrocytes are seen in patients with myelofibrosis; however, they would not be seen in G6PD deficiency.

Bullet Summary:
Primaquine prophylaxis of malaria can lead to hemolytic anemia in patients with G6PD deficiency.