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

ABSTRACT

Introduction

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.

Areas covered

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.

Expert opinion

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.

KEYWORDS:

• Hematopoietic cell transplantation
• malignancy
• primary immunodeficiency

Article highlights

• Secondary malignancy post HCT for malignant disorders is well recognized and long-term follow including screening for malignancy is well established.

• Patients with PID are at risk of developing malignancy as part of their underlying disorder. The most common form of malignancy is lymphoma. Survival from HCT for PID has improved dramatically in recent years. The risk of developing malignancy after successful HCT is less clear.

• The risk of developing malignancy post HCT is dependent on patient-, disease- and transplant-specific factors. Some disorders are associated with specific malignancies for which the risk is not altered by changing the hematopoietic cell lineages. Age at HCT, chemotherapy agents and other drugs used, radiation, occurrence graft-versus-host disease, infections, and degree of donor chimerism may all have a role in carcinogenesis.

• PID transplant survivors represent a unique cohort compared to transplant survivors of malignant and non-malignant hematological disorders.

Declaration of interest

M Slatter has received honoraria from MEDAC Pharmaceuticals for speaking. The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.