Clinical Spectrum of Hemolytic Anemia Associated with Glucose-6-Phosphate Dehydrogenase Deficiency

Abstract

Patients (102) with glucose-6-phosphate dehydrogenase (G-6PD) deficiency were studied to determine the characteristics of the associated hemolytic anemia. Of 119 acute episodes of hemolysis, 73 were related only to concurrent illness and 46 to administration of chemotherapeutic agents. Twenty chronic episodes (from 2 months to 30 years) were evenly divided between those related to intermittent or continued drug therapy and those to chronic illness alone. There were 2 instances of congenital non-spherocytic hemolytic anemia. An additive hemolytic effect was noted when 2 precipitating stimuli were present simultaneously. Thirty % of the patients with hemolytic episodes were Caucasian, a frequency higher than expected on the basis of the frequency of the trait in the clinic population. Forty-two % of the patients with hemolytic episodes were females, suggesting that clinically apparent anemia occurs when the trait is present in the heterozygous state. There is a wide variation in the severity of the clinical episodes.