G6PD deficiency is a defect in the G6PD enzyme, which provides protection against oxidative stress in red blood cells. It is an X-linked inherited condition, though female heterozygotes have some protection against malaria. Those with G6PD deficiency experience hemolytic anemia during times of oxidative stress caused by factors like infections, medications, or foods like fava beans. The deficiency results in inadequate levels of NADPH and glutathione, leaving red blood cells vulnerable to damage and hemolysis. Symptoms of the acute hemolytic anemia appear 24-48 hours after exposure to the triggering agent. Laboratory tests show signs of hemolysis and low G6PD enzyme activity. There is no cure or treatment other than

1. GIucose-6-phosphate dehydrogenase deficiency
Hemolytic anemia Defective red cell metabolism
Developed by-Dr.Abdulrazzaq Othman Alagbare
M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
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2. Defective red cell metabolism
ANEMIA -Dr.Alagbare
• G6PD is a main enzyme of RBC
• G6PD provide 90% of energy for the RBC
• G6PD inherited by X-linked
• The female heterozygotes have an advantage of resistance to
Falciparum malaria.
GIucose-6-phosphate dehydrogenase deficiency

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FUNCTION OF G6PD
• Regenerates NADPH, allowing regeneration of glutathione
• Protects against oxidative stress
• Lack of G6PD leads to hemolysis during oxidative stress- infection, medication, fava beans

4. • 1-sever 50-70% G6PD is absent
• 2-mild 25-35 % G6PD is absent
• It is acute intravascular hemolytic anemia
• 400 type of G6PD deficiency
• 400 million person are suffering in the world
Types of G6PD deficiency:
Causes of G6PD deficiency absent or decreased function of a
metabolic enzyme (G6PD), usually in male, Rare in female
ANEMIA -Dr.Alagbare

5. GIucose-6-phosphate dehydrogenase deficiency
Mechanism :
Inadequate amounts of GSH lead to  Decreased NADPH generation which
protect the Fe2- hemoglobin inside the RBC  lead to produce methemoglobin
and forming little inclusions known as the “Heinz bodies, Which removed by
spleen (Bite and Blister cells)
ANEMIA -Dr.Alagbare

6. ANEMIA -Dr.Alagbare
G6PD - Risk factors
The RBC affected and destroyed if the patient exposed to
• 1-fava beans
• 2-drugs
• 3-infection
• 4-chemicals
The symptoms appears after 24 to 48hrs post exposed to caused
agent

7. Cont. GIucose-6-phosphate dehydrogenase deficiency
Lab's Findings: CBC:
• Sever anemia with Hb 4-6g/dl PCV and RBC count are decreased
• WBC: increased (in many cases)
ANEMIA -Dr.Alagbare

8. Cont. GIucose-6-phosphate dehydrogenase deficiency
Blood film study – G6PD Deficiency. The RBC has
Heinz bodies, Basophilic stippling Blister cells Bite cells polychromasia
ANEMIA -Dr.Alagbare
Urine : brown color with hemosiderinuria

9. Cont. GIucose-6-phosphate dehydrogenase deficiency
Diagnostic test of G6PD:
• Measure the amount of the G6PD
• Measure the G6PD activity
ANEMIA -Dr.Alagbare
Performing assays for G-6-PD during hemolysis and reticulocytosis give
false result, the test must be done after one month of the hemolytic
eipsode

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11. ‫رب‬ ‫هلل‬ ‫الحمد‬
‫العالمين‬
‫وجه‬ ‫ابتغاء‬ ‫الجهد‬ ‫هذا‬
‫الكريم‬ ‫هللا‬