Description

Patients with deficiency of glucose-6-phosphate dehydrogenase (G6PD) enzyme activity in erythrocytes may be unable to generate sufficient NADPH to compensate for a significant oxidative stress, which can result in hemolysis. Some patients with G6PD dehydrogenase may develop an acute hemolytic anemia after ingestion of fava beans (broadbean).


 

Clinical findings:

(1) variable history of G6PD deficiency

(2) history of recent fava bean ingestion

(3) malaise

(4) general weakness and lethargy

(5) nausea and vomiting

(6) headache

(7) abdominal or lumbar pain

(8) chills and/or tremors

(9) fever

(10) hemoglobinuria for several days

(11) jaundice for several days

(12) variable hepatosplenomegaly

(13) no enzymatic evidence of hepatitis

(14) no evidence of acute gallstone or biliary disease (patients with chronic hemolytic anemia may be at increased risk for gallstones)

(15) anemia with reticulocytosis

 

Since the erythrocytes that hemolyze are older erythrocytes with the lowest levels of G6PD, laboratory tests for G6PD activity done during or soon after a hemolytic episode will often be normal or mildly depressed. If G6PD deficiency is suspected then testing should be done several weeks after the last hemolytic episode.

 


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