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Favism: disordered erythrocyte calcium homeostasis
A De Flora, U Benatti, L Guida, G Forteleoni and T Meloni
The biochemical events that take place during acute hemolysis of G6PD-
deficient subjects in favism are far from being elucidated. Evidence is
here reported for a constantly and heavily disordered calcium homeostasis
in the erythrocytes from seven favic patients. The abnormality, ie, a
significantly impaired calcium ATPase activity and a parallel marked
increase of intracellular calcium levels, was characteristic of the acute
hemolytic crisis although unrelated to the attendant reticulocytosis.
Concomitantly, a remarkable decrease of intracellular potassium was also
observed. The mean +/- SD Ca2+-ATPase activity in the favic patients was
20.8 +/- 7.8 mumol Pi/g Hb/h compared with 37.2 +/- 8.5 in the matched
controls represented by 12 healthy G6PD-deficient subjects (P less than
.001). The mean +/- SD intraerythrocytic calcium content was 288 +/- 158
mumol/L of erythrocytes in the favic patients as compared with 22.0 +/- 8.2
in the G6PD-deficient controls (P less than .001). The intraerythrocytic
potassium content was 76.6 +/- 19.3 mmol/L of erythrocytes in the favic
patients and 106.6 +/- 8.2 in the G6PD-deficient controls (P less than
.001). In vitro incubation of normal and G6PD-deficient erythrocytes with
divicine, a pyrimidine aglycone present in fava beans and strongly
implicated in the pathogenesis of favism, reproduces most of these events,
including drop of calcium ATPase, increased intracellular calcium, and
leakage of erythrocyte potassium.
Volume 66,
Issue 2,
pp. 294-297,
08/01/1985
Copyright © 1985 by The American Society of Hematology
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