Sickle cell anaemia and bone marrow transplantation

Abstract

Sickle cell anaemia is an hereditary autosomal recessive disorder characterized by chronic haemolytic anaemia, painful episodes due to the occlusion of small vessels and an increased susceptibility to severe, often fatal infections. It is a disease commonly seen in equatorial Africa, particularly in the west, where 40 to 50% of the members of certain ethnic groups are carriers of the sickle cell gene. The gene is not restricted to blacks however, as it appears in parts of Turkey, Saudi Arabia, southern India, Sicily, Cyprus and Greece. Thirty years ago, most internists would have been surprised when they encountered an adult with sickle cell anaemia. At the present time, early diagnosis and comprehensive supportive care result in a better survival, although morbidity from vaso-occlusive crises and mortality from progressive organ failure persist especially for people living in third world countries.The aim of this work is to summarise the clinical problems encountered by these patients, to try to delineate factors influencing the natural course of the disease, to study the therapeutical options availed, and mainly to examine the value of bone marrow transplantation in this conditionThèse d'agrégation de l'enseignement supérieur -- UCL, 199