چكيده لاتين :
Primary Immunodeficiencies constitute a group of highly complex congenital
disorders most of which are characterized by a very poor prognosis. Allogeneic
hematopoietic stem cell transplantation (HSCT) has become an established curative
treatment approach in many of these disorders, which may be permanently corrected.
In this presentation basic and practical aspects of HSCT are presented, with an
emphasis on its application in lymphocyte disorders such as severe combined
immunodeficiency (SCID). Optimal results and outcome of HSCT are highly
dependant on early and correct diagnosis of these rare disorders, and HSCT should
usually be applied early in the course of the disease in order to prevent irreversible
complications from infections. Clinical results will be summarized based on recent
analysis performed in large patient cohorts, which have shown steady improvements
and have led to a marked change in the prognosis of patients with primary
immunodeficiencies.
