原发性免疫缺陷病是一类罕见的单基因遗传病，通常临床表现较重，致死率高。能否早期诊断并及时有效 地完成免疫重建是影响预后的关键。在基因治疗尚在试验阶段的今天，造血干细胞移植是目前重建免疫的唯一手段。去 αβT/B细胞的造血干细胞移植作为一种新型的去T细胞移植策略，近年来逐渐被用于原发性免疫缺陷病患儿的根治治 疗，并在防控移植物抗宿主病、减少移植后感染等方面取得了令人满意的效果。

Primary immunodeficiency disease (PID) is a rare monogenic hereditary disease with severe clinical manifestations and high mortality. Early diagnosis and complete immune reconstitution in a timely and effective manner is the key to prognosis. Nowadays, gene therapy is still in the experimental stage, and hematopoietic stem cell transplantation is currently the only means of reconstructing immunity. TCRαβ T cell/B cell-depleted hematopoietic stem cell transplantation as a novel T-cell-free transplantation strategy has been gradually used in the radical treatment of primary immunodeficiency disease in children in recent years. It has achieved satisfactory results in the prevention and control of graft-versus-host disease and the reduction of post-transplant infection. This article reviews its biological basis and recent progress.