Studies on glutathione stability in erythrocytes of cases with past history of favism or sulfa-drug-induced hemolysis

1. The glutathione stability test of Beutler has been performed on bloodsamples from cases with past history of hemolytic anemia due to Vicia fabaor sulfa drugs and some members of their families.

2. In all the affected cases the level of reduced glutathione (GSH) inerythrocytes fell below 30 mg./100 cc. RBC after two hours incubation withacetylphenylhydrazine (APH). Four out of 13 relatives examined showedthe same results.

3. Out of 117 control cases only five showed similar results. All the fivecases were Jews of Sefardic origin.

4. Indications have been found that by addition of glucose to the bloodsamples tested by the above method more clear-cut results could be obtained.

5. The decrease of the reduced glutathione level in the abnormal cells incubated with acetylphenylhydrazine could not be prevented by addition ofglucose.

6. The decrease of reduced glutathione level in normal and abnormal cellsincubated at 37 C. for 24 hours without acetylphenylhydrazine was preventedby addition of glucose. No such protective action of glucose on GSH inhemolysates had been found.

Submitted on August 7, 1957 Accepted on November 18, 1957     

Studies on erthrocytes in cases with past history of favism and drug-induced acute hemolytic anemia

Studies have been carried out on erythrocytes of cases with past history ofacute hemolysis due to Favism, sulpha drugs or PAS.

No indication was found of abnormal osmotic or mechanical fragility, electrophoretic mobility of hemoglobin or its amino acid content. Similarly no abnormal glycolytic or catalase activities were detected.

Glutathione (GSH) deficiency of erythrocytes was discovered in all casesstudied. Similarly, glutathione (GSH) deficiency was a frequent finding amongthe relatives of affected cases.

No such glutathione deficiency could be established in the cases of thalassemiamajor so far studied, though both patient-groups without any exception belongto the same ethnographic denomination (non-Ashkenazic).

This investigation was aided by a grant from the Israel Research Council.

Submitted on October 10, 1956 Accepted on November 26, 1956