Hemolysis in glucose-6-phosphate dehydrogenase

Figure 11-8. Mechanism of hemolysis in glucose 6-phosphate dehydrogenase deficiency.

In some drug reactions, several of these hypersensitivity responses may present simultaneously. Some adverse reactions to drugs may be mistakenly classified as allergic or immune when they are actually genetic deficiency states or are idiosyncratic and not mediated by immune mechanisms (eg, hemolysis due to primaquine in glucose-6-phosphate dehydrogenase deficiency, or aplastic anemia caused by chloramphenicol). 

In the historical introduction to this book (Chapter 1, Sec. 2.1), I mentioned primaquine hemolysis and Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency during the second world war in American soldiers. I did not... 

A peripheral eosinophilia is usual (2). Hemolysis has been reported once in glucose-6-phosphate dehydrogenase (G6PD) deficiency (SED-8, 691) (9). 

Primaquine is the prototypical drug that induces hemolysis in persons deficient in glucose-6-phosphate dehydrogenase. It may also occur, less frequently, during treatment with chloroquine or quinine. The answer is (D). 

Water-soluble menadione can produce anemia, skin irritation, polycythemia, splenomegaly, renal and hepatic damage, and death. Also implicated in hemolytic anemia, hyperbilirubinemia, and kernicterus. Menadione can induce erythrocyte hemolysis in individuals with genetic defect in glucose-6-phosphate dehydrogenase. 

Glucose- 6-phosphate dehydrogenase Low or absent enzyme activity in about 10% of African populations. Hemolysis following intake of a number of drugs which have electrophilic reactive metabolites, but also, carriers of this enzyme deficiency have a partial protection from malaria. 

Genetic deficiency of glucose-6-phosphate dehydrogenase, with consequent impairment of the generation of NADPH, is common in populations of Mediterranean and Afro-Caribbean origin. The defect is manifested as red cell hemolysis (hemolytic anemia) when susceptible individuals are subjected to oxidants, such as the an-timalarial primaquine, aspirin, or sulfonamides or when... 

The answers are 484-k 485-j. (tlardman, pp 1061-1062, 1682-1685.) Sulfonamides can cause acute hemolytic anemia. In some patients it mayr be related to a sensitization phenomenon, and in other patients the hemolysis is due to a glucose-6-phosphate dehydrogenase deficiency Sulfamethoxazole alone or in combination with trimethoprim is used to treat UTls. The sulfonamide sulfasalazine is employed in the treatment of ulcerative colitis. Daps one, a drug that is used in the treatment of leprosy, and primaquine, an anti mala rial agent, can produce hemolysis, particularly in patients with a glucose-6-phosphate dehydrogenase deficiency. 

In a fatal human exposure, a worker engaged in emptying metal gas cylinders of methyl mercaptan was found comatose at the work site he developed expiratory wheezes, elevated blood pressure, tachycardia, and marked rigidity of extremities. Methemoglobinemia and severe hemolytic anemia developed with hematuria and proteinuria but were brief in duration deep coma persisted until death due to pulmonary embolus 28 days after exposure. It was determined that the individual was deficient in erythrocyte glucose-6-phosphate dehydrogenase, which was the likely cause of the hemolysis and formation of methemoglobin. 

Adverse reactions occur more frequently in slow acetylators. They include acute hemolysis in patients with glucose-6-phosphate dehydrogenase deficiency, and agranulocytosis. Fever, arthralgias, and rashes occur in up to 20% of patients. Gastrointestinal complaints are common. Hypersensitivity reactions including photosensitivity are also seen. Less frequent are hepatic function disturbances. 

Some adverse reactions are unrelated to either allergy or overdose these are termed idiosyncratic. For instance, sulfonamides may precipitate acute hemolysis in some people having a glucose-6-phosphate dehydrogenase dehciency. 

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