Clinical and Biochemical Data

In Greece, favism is the most common type of acute haemolytic anaemia in childhood (Kattamis, unpublished). The disease seems to be restricted mainly to certain Mediterranean countries (Sardinia, Greece, and Israel, especially among Sephardic Jews); lately, it has also been described in other areas of the world (Motulsky and Stamatoyannopoulos, 1966). Many aspects of the pathogenesis of favism have been elucidated in the past decade through the evolutionary progress in the study of G6PD deficiency (Beutler, 1965; Motulsky, 1965), and it was shown that favism affected only individuals with G6PD deficiency (Szeinberg et al., 1957; Sansone, Piga, and Segni, 1958; Zannos-Mariolea and Kattamis, 1961). This red cell enzymatic defect is inherited as a sex-linked character; it exists in a number of variants, and is also related to the haemolytic syndrome which follows primaquine and other drug administration (Tarlov et al., 1962), severe neonatal jaundice unrelated to rhesus or ABO incompatibility (Fessas, Doxiadis, and Valaes, 1962), congenital type I non-spherocytic haemolytic anaemia and haemolytic anaemia due to infection (Choremis et al., 1966; Kattamis and Kyriazakou, 1966). Until now favism has been described only in individuals with the common Mediterranean type of G6PD deficiency, which is widely, though unevenly, distributed in Greece (Choremis, ZannosMariolea, and Kattamis, 1962; Choremis et al., 1963a, Allison et al., 1963; Stamatoyannopoulos, Panayotopoulos, and Motulsky, 1966b). The pathogenesis of favism, though directly related to the Mediterranean type of deficiency, is still obscure. Accumulating data suggest that additional factors, probably genetic, are required for the development of this peculiar haemolytic syn-