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.

中文翻译：

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原发性免疫缺陷患者的恶性肿瘤造血后干细胞移植。

简介

造血细胞移植 (HCT) 是一种治疗越来越多的原发性免疫缺陷 (PID) 的方法。PID 患者的恶性肿瘤比一般人群更常见，本综述将讨论成功的 HCT 是否有望消除或改变这种风险。众所周知，恶性疾病的 HCT 后会发生第二次恶性肿瘤，但在因 PID 移植的患者中通常不太预期。

覆盖区域

本文回顾了最近发表的文献，重点关注 PID 儿童的恶性肿瘤模式、发病率和 PID 后 HCT 发生恶性肿瘤的风险因素以及降低风险的可能策略。

专家意见

在过去的 20 年里，PID 的 HCT 后生存率有了显着提高，基因组革命导致了越来越多的适应症。为了提高长期生活质量，需要关注晚期效应，包括恶性肿瘤发生频率高于一般人群预期的可能性，了解风险并改进移植过程以将其最小化。需要进一步研究。