No abstract

Summary: The rate of globin chain production has been studied in patients with homozygous β‐thalassaemia, heterozygous β‐thalassaemia, haemoglobin E‐thalassaemia, and sickle‐cell‐thalassaemia, and compared with that in non‐thalassaemic individuals. A partial or total deficit of β‐chain synthesis has been demonstrated in all forms of β‐thalassaemia. This results in the production of a large intracellular pool of α‐chains, the kinetics of which have been worked out. The α‐chains in this pool appear to contain haem and are unstable, rapidly becoming associated with the stromal fraction. These findings are examined in terms of the pathogenesis of the anaemia of thalassaemia.

Studies on the treatment and prevention of iron deficiency anemia, in pregnant and nonpregnant women and in men, were conducted in Thailand and Burma. The effects of the dose of Fe, duration of Fe administration, additional supplementation with folate, mode of supplement delivery (either supervised or unsupervised), and the presence of Hb(AE) were studied. The frequency and severity of side effects were also recorded. Fe administration resulted in an increase in hemoglobin concentration in all anemic individuals but approximately 20% failed to reach normality. The length of administration and the dose influenced the results. Frequency and severity of side effects increased with the dose of Fe administered. Folate supplementation did not affect the results. It appears possible to integrate a program of prevention and treatment of Fe deficiency anemia in a primary health-care system but the constraints and limitations of achievable results should be recognized.

Eighty-five cases of PNH in Thailand were analysed, with emphasis on the comparison with European series and on an association with aplastic anaemia. Compared with European series, two points emerge: (1) the disease in Thai affects individuals of younger age group with a tendency to male preponderance; (2) the incidence of arterial and venous thrombosis is relatively rare. In association with aplastic anaemia, two main categories of PNH patients, are described, which differ in their clinical and laboratory citeria.