Glucose-6-phosphate deficiency: Difference between revisions
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**Leads to removal of the cell from circulation via the spleen | **Leads to removal of the cell from circulation via the spleen | ||
==Clinical== | ==Precipitants== | ||
*Infection | |||
*Fava Beans | |||
*Medications | |||
**[[Nitrofurantoin]] | |||
**[[Phenazopyridine]] | |||
**[[Dapsone]] | |||
**[[Chloramphenicol]] | |||
**[[Antimalarials]] | |||
**[[Sulfonamides]] | |||
**[[Ciprofloxacin]], norfloxacin | |||
**Methylene blue | |||
**Vitamin K analogues | |||
==Clinical Features== | |||
*Fatigue | *Fatigue | ||
*Hemolytic anemia | *Hemolytic anemia | ||
*Jaundice | *[[Jaundice]] | ||
*Splenomegaly | *[[Splenomegaly]] | ||
== | ==Differential Diagnosis== | ||
{{Anemia DDX}} | |||
== | ==Evaluation== | ||
===Workup=== | |||
*CBC | *CBC | ||
**Heinz Bodies | **Heinz Bodies | ||
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**Retic count high | **Retic count high | ||
***Coombs negative: G6PD, SCD, spherocytosis, microangiopathic hemolysis | ***Coombs negative: G6PD, SCD, spherocytosis, microangiopathic hemolysis | ||
==Management<ref>Schick P et al. eMedicine. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management. Sep 29, 2015. http://emedicine.medscape.com/article/200390-treatment#showall</ref>== | ==Management<ref>Schick P et al. eMedicine. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management. Sep 29, 2015. http://emedicine.medscape.com/article/200390-treatment#showall</ref>== | ||
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**Exchange transfusion for severe neonatal jaundice | **Exchange transfusion for severe neonatal jaundice | ||
== | ===Alternative Antibiotics=== | ||
* | *Cephalexin (Keflex) | ||
==Disposition== | |||
== | ==Complications== | ||
* | *Severe hemolysis and anemia | ||
*Cardiovascular collapse | |||
==Also See== | ==Also See== | ||
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==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Revision as of 02:57, 18 August 2016
Background
- X-Linked recessive; protects against Malaria
- African, Asian, and Mediterranean descent
- Nonimmune mediated hemolytic anemia
- Stress or drugs can cause hemoglobin precipitation within the RBC
- Leads to removal of the cell from circulation via the spleen
Precipitants
- Infection
- Fava Beans
- Medications
- Nitrofurantoin
- Phenazopyridine
- Dapsone
- Chloramphenicol
- Antimalarials
- Sulfonamides
- Ciprofloxacin, norfloxacin
- Methylene blue
- Vitamin K analogues
Clinical Features
- Fatigue
- Hemolytic anemia
- Jaundice
- Splenomegaly
Differential Diagnosis
Anemia
RBC Loss
RBC consumption (Destruction/hemolytic)
- Hereditary
- Acquired
- Microangiopathic Hemolytic Anemia (MAHA)
- Autoimmune hemolytic anemia
Impaired Production (Hypochromic/microcytic)
- Iron deficiency
- Anemia of chronic disease
- Thalassemia
- Sideroblastic anemia
Aplastic/myelodysplastic (normocytic)
Megaloblastic (macrocytic)
- Vitamin B12/folate deficiency
- Drugs (chemo)
- HIV
Evaluation
Workup
- CBC
- Heinz Bodies
- Retic Count
- Retic count high
- Coombs negative: G6PD, SCD, spherocytosis, microangiopathic hemolysis
- Retic count high
Management[1]
- Identify and discontinue precipitating agent
- Supportive care for anemia, with transfusions rarely needed
- Hemolysis usually self-limited, resolving within 8-14 days
- Infants
- Prolonged neonatal jaundice due to G6PD deficiency may require phototherapy
- Exchange transfusion for severe neonatal jaundice
Alternative Antibiotics
- Cephalexin (Keflex)
Disposition
Complications
- Severe hemolysis and anemia
- Cardiovascular collapse
Also See
References
- ↑ Schick P et al. eMedicine. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management. Sep 29, 2015. http://emedicine.medscape.com/article/200390-treatment#showall