Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

Epidemiology

Demographics/Prevalence

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Occurs with Increased Incidence in African-Americans, Mediterranean Descent, Southeast Asians

Diagnosis

Blood Glucose-6-Phosphate Dehydrogenase (G6PD) (see Glucose-6 Phosphate Dehydrogenase, Glucose-6 Phosphate Dehydrogenase)

Technique
- Whole Blood Sample: collected in lavender EDTA top tube, green top heparinized tube, and yellow top ACD tube
- Sample Must Not Be Hemolyzed
Interpretation
- Decreased

Nitroblue Tetrazolium Test (NBT) (see Nitroblue Tetrazolium Test, Nitroblue Tetrazolium Test)

May Be Abnormal in Very Severe G6PD Deficiency
- This is a Test Which is Abnormal and Used to Diagnose Chronic Granulomatous Disease (CGD) (see Chronic Granulomatous Disease, Chronic Granulomatous Disease)

Clinical Manifestations

Hematologic Manifestations

Hemolytic Anemia (see Hemolytic Anemia, Hemolytic Anemia)

Drugs Which May Trigger Hemolysis in Patients with G6PD Deficiency
- Definite Risk
  - Acetanilide (Acetanilid, Antifebrin) (see Acetanilide, Acetanilide)
  - Dapsone (see Dapsone, Dapsone): sulfone with a similar mechanism of action that of the sulfonamides
  - Dapsone/Chlorproguanil (Lapdap)
  - Methylene Blue (see Methylene Blue, Methylene Blue)
  - Nalidixic Acid (see Nalidixic Acid, Nalidixic Acid)
  - Naphthalene (see Naphthalene, Naphthalene)
  - Niridazole
  - Nitrofurantoin (see Nitrofurantoin, Nitrofurantoin)
  - Phenazopyridine (see Phenazopyridine, Phenazopyridine)
  - Sulfonamides (see Sulfonamides, Sulfonamides)
    - Sulfamethoxazole-Trimethoprim (see Sulfamethoxazole-Trimethoprim, Sulfamethoxazole-Trimethoprim): rarely causes hemolysis in setting of G6PD deficiency
    - Other Sulfonamides
  - Primaquine (see Primaquine, Primaquine): can cause hemolysis in G6PD deficiency
- Possible Risk
  - Acetylsalicylic Acid (Aspirin) (see Acetylsalicylic Acid, Acetylsalicylic Acid): at high doses (>3g/day)
  - Chloroquine (see Chloroquine, Chloroquine)
  - Ciprofloxacin (Cipro) (see Ciprofloxacin, Ciprofloxacin)
  - Norfloxacin (see Norfloxacin, Norfloxacin)
  - Rasburicase (Elitek) (see Rasburicase, Rasburicase)
  - Sulfonamides (see Sulfonamides, Sulfonamides)
    - Sulfamidine
    - Sulfasalazine (Azulfidine) (see Sulfasalazine, Sulfasalazine)
  - Vitamin C (Ascorbic Acid) (see Vitamin C, Vitamin C): at doses >1g/day
  - Vitamin K Analogues
- Doubtful Risk
  - Acetaminophen (Tylenol) (see Acetaminophen, Acetaminophen)
  - Acetylsalicylic Acid (see Acetylsalicylic Acid, Acetylsalicylic Acid): at lower doses (<3g/day)
  - Chloramphenicol (see Chloramphenicol, Chloramphenicol)
  - Doxorubicin (Adriamycin) (see Doxorubicin, Doxorubicin)
  - p-Aminosalicylic Acid
  - Phenacetin (see Phenacetin, Phenacetin)
  - Quinine (see Quinine, Quinine)
  - Sulfonamides (see Sulfonamides, Sulfonamides)
    - Probenecid (see Probenecid, Probenecid)
    - Sulfadiazine (see Silver Sulfadiazine, Silver Sulfadiazine)
    - Sulfisoxazole

Other Manifestations

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Treatment

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References

Methemoglobinemia and hemolytic anemia after rasburicase administration in a child with leukemia. Int J Clin Pharm. 2011 Feb;33(1):58-60. doi: 10.1007/s11096-011-9484-3. Epub 2011 Feb 4 [MEDLINE]
Rasburicase causing severe oxidative hemolysis and methemoglobinemia in a patient with previously unrecognized glucose-6-phosphate dehydrogenase deficiency. Acta Haematol. 2013;130(4):254-9. doi: 10.1159/000351048. Epub 2013 Jul 11 [MEDLINE]