[Beta-thalassemia in metropolitan France]

Abstract

Objectives: Update the data collected in 1990 in order to assess the distribution and management of thalassemic patients presently living in Metropolitan France.

Methods: A survey conducted in France in the clinical and biological departments of haematology permitted collection of epidemiological, clinical and biological data in a population of thalassemic patients followed-up in metropolitan France.

Results: Analysis of the replies revealed a total of 362 thalassemia with 249 beta-thalassemia major, 81 beta-thalassemia intermedia and 32 E-beta thalassemia. These patients predominated in the East of France and in the large cities. The total number of patients has remained stable over the last decade and new cases are decreasing. Among the 249 patients presenting with a ss-thalassemia major, 42 had received a bone marrow graft, whereas 207 were systematically transfused and 189 regularly underwent iron chelating.

Comments: Management is standardised and efficient but could be improved on with regard to iron chelating therapy.