Glucose 6 Phosphate Dehydrogenase Deficiency

X-linked recessive disorder
Diminished G6PD activity impairs the ability of the cell to form the NADPH that is essential for the maintenance of the reduced glutathione pool. This results in a decrease in the cellular detoxification of free radicals and peroxides formed within the cell.
Most severe in erythrocytes, where the pentose phosphate pathway provides the only means of generating NADPH.
Glutathione also helps maintain the reduced states of sulfhydryl groups in proteins, including hemoglobin.

Heinz Bodies Exposure of G6PD-deficient red cells to high levels of oxidants causes the cross-linking of reactive sulfhydryl groups on globin chains, which become denatured and form membrane-bound precipitates known as Heinz bodies. These are seen as dark inclusions within red cells stained with crystal violet.
Heinz bodies can damage the membrane sufficiently to cause intravascular hemolysis.
As inclusion-bearing red cells pass through the splenic cords, macrophages pluck out the Heinz bodies and create "Bite Cells".

Precipitating factors in G6PD deficiency

Patients with G6PD deficiency develop hemolytic anemia if they are treated with an oxidant drug, ingest fava beans, or contract a severe infection.
Oxidant drugs: mnemonic AAA—

Antibiotics (for example, sulfamethoxazole and chloramphenicol),
Antimalarials (for example, primaquine but not quinine), and
Antipyretics (for example, acetanilid but not acetaminophen).

Favism: G6PD deficiency is susceptible to the hemolytic effect of the fava bean, a dietary staple in the Mediterranean region. All patients with favism have G6PD deficiency.
Infection

Neonatal jaundice: Babies with G6PD deficiency may experience neonatal jaundice appearing one to four days after birth. The jaundice, which may be severe, typically results from increased production of bilirubin.

Links to:

Hereditary Spherocytosis	Sickle Cell Anemia
Thalassemias	Immunohemolytic anemias
Hemolytic Anemias Introduction	Anemia

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