G6pd deficiency for Lab.students

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