Thalassaemia Types among Kurdish Jews in Israel

The existence of thalassaemia among Jews of the Kurdish community is, by now, well established. Among the earlier reports were those of Schieber (1945), Matoth, Shamir and Freundlich (1955) and Dreyfuss (1955). Several estimates of the thalassaemia gene frequency in this community have been made but none of these took account of the more recent classification of thalassaemia types (Ingram and Stretton, 1959; Huehns and Shooter, 1965). The main distinction is between α‐ and β‐thalassaemias. Although the clinical signs and many haematological deviations are similar in α‐ and β‐trait carriers, the haemoglobin A2 fraction is increased in β‐thalassaemia, but normal or decreased in α‐thalassaemia trait. Moreover, part of the α‐trait carriers have been reported to display inclusion bodies in their red cells after staining with Brilliant Cresyl Blue (Fessas, 1963). Matoth and Pinhas (1958) gave a gene frequency estimate of 0.066 for Kurdish Jews, which was based on the incidence of thalassaemia major in the community. This estimate applies only to the β‐thalassaemia gene(s) and disregards the possible existence of α‐thalassaemia. Cohen, Goldschmidt, Ada, Matoth, Theodor and Szabo (1959) screened families of Kurdish Jews for iron‐resistant hypochromic and microcytic anaemia. They arrived at an estimate of 0.25 for the frequency of thalassaemia heterozygotes, including probably the majority of α‐ as well as β‐trait carriers without distinction between them. Ramot, Abrahamov, Freier and Gafni (1964) observed a slightly lower heterozygote frequency of 0.20. Their screening method was based on elevated levels of Hb A2 and of foetal haemoglobin and probably detected only a small fraction of α‐thalassaemia trait carriers. Haemoglobin H disease was reported in several Israeli families from various Oriental countries (Ramot, Sheba, Fisher, Ager and Lehmann, 1959; Klibansky, Djaldetti, Joshua and De Vries, 1960; Zlotnik, Ramot and Hamosh, 1964). Among these, only the kinship described by Zlotnik et al. (1964) has an affinity to the Kurdish community, since some of its members originated from Urfa in Eastern Turkey. The aim of the present survey was to detect the different thalassaemia types existing in the community of Kurdish Jews, to study any possible interaction between them and to estimate their relative, though not their absolute, frequencies.