Thalassemic syndromes in Latium: epidemiological evaluation

Abstract

BACKGROUND AND OBJECTIVE: After the first National Census of transfusion-dependent thalassemics (1984), in 1992 the Italian Association of Pediatric Hematology and Oncology and the National Health Institute organized its 3rd edition. Here, results concerning Latium are presented and discussed. DESIGN AND METHODS: Data for Latium, as in the rest of Italy, were collected by a single reference center; among all eventual care centers for thalassemia initially contacted, only those with patients were sent data forms. For new cases, a questionnaire was submitted to parents to obtaining social data, and information on their knowledge of thalassemia. RESULTS: Collected data were divided into 2 groups: old cases, before June 30, 1988, and new cases, between July 1, 1988 and December 31, 1992. On the whole, 262 transfusion-dependent thalassemics (127 m., 135 f.) could be counted. Sixteen percent were affected with thalassemia intermedia, severe enough as to need regular transfusions. New patients (last 5 years): the birth of almost 80% of them was due to combined mistakes of parents and doctors. Disease evolution: 19/262 patients had been submitted to BMT (presently transfusion-free). Causes of death: 22 patients died in the considered period, mostly for cardiologic complications. INTERPRETATION AND CONCLUSIONS: Data emerging from censuses on specific pathologies of high social impact (such as thalassemia) may help health plans to rationalize public expenditure, especially by improving working conditions of care centers.