Malignancy post-hematopoietic stem cell transplant in patients with primary immunodeficiency

Hematopoietic cell transplantation (HCT) is a curative treatment for an expanding number of primary immunodeficiencies (PIDs). Malignancies are more common in patients with PID than in the general population, and this review will discuss whether a successful HCT is expected to abolish or alter this risk. Second malignancy post HCT for a malignant disease is well known to occur, but generally less expected in patients transplanted for PID.

This article reviews recently published literature focusing on the pattern of malignancy in children with PID, incidence, and risk factors for developing malignancy post-HCT for PID and possible strategies to reduce the risks.

Survival post HCT for PID has improved dramatically in the last 20 years and the genomic revolution has led to an expanding number of indications. To improve long-term quality of life attention needs to focus on late effects, including the possibility of malignancy occurring more frequently than expected in the general population, understand the risks and improve the process of transplantation in order to minimize them. Further studies are needed.