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Observational Study
. 2025 Sep;156(3):825-834.
doi: 10.1016/j.jaci.2025.04.032. Epub 2025 May 14.

The impact of allogeneic hematopoietic stem cell transplantation on pulmonary complications in adults with inborn errors of immunity

Aurélie Le Gal  1 , Ambroise Marçais  2 , Céline Goyard  1 , Anne-Laure Brun  3 , Morgane Cheminant  2 , Nizar Mahlaoui  4 , Claire Givel  1 , Colas Tcherakian  1 , Alexandre Chabrol  1 , Frédéric Wallyn  5 , Leonardo Magro  6 , Flore Sicre de Fontbrune  7 , Regis Peffault de la Tour  7 , Abdellatif Tazi  8 , Amira Benattia  9 , Remi Valter  10 , Philippe Devillier  11 , Louis-Jean Couderc  12 , Felipe Suarez  13 , Emilie Catherinot  1 , Hélène Salvator  14
Affiliations
Free article
Observational Study

The impact of allogeneic hematopoietic stem cell transplantation on pulmonary complications in adults with inborn errors of immunity

Aurélie Le Gal et al. J Allergy Clin Immunol. 2025 Sep.
Free article

Abstract

Background: Pulmonary involvement (repeated lung infections, lung parenchymal inflammation, scarring, and malignancies) is frequent in patients with inborn errors of immunity (IEIs) and accounts for a significant proportion of the disease burden. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) can cure most severe IEIs. The indications for allo-HSCT have recently been extended to adults.

Objective: We sought to assess the impact of allo-HSCT specifically on respiratory status.

Methods: We retrospectively analyzed data of 50 patients with IEIs who underwent a first allo-HSCT after the age of 16 at 3 expert centers in France.

Results: The median length of follow-up was 4.8 years (interquartile range: 1.6-9.2) before allo-HSCT and 3 years (interquartile range: 1.4-6.0) after allo-HSCT. Ten patients died as a result of allo-HSCT-related complications. Four patients developed bronchiolitis obliterans syndrome. After 1-year posttransplantation, the mean annualized rate of severe respiratory infections (0.14 [95% CI: 0.04 to 0.24]) was lower than the value recorded before transplantation (0.54 [95% CI: 0.25 to 0.82]; P = .003 for paired comparisons of equivalent durations). Lung function was declining before allo-HSCT (mean FEV1: -2.09% predicted/year [95% CI: -7.27 to 3.09]) but increased afterward (+2.44% predicted/year [95% CI: -4.79 to 9.69], P = .0034 for paired comparisons). On computed tomography scans of the chest, bronchial disorders and lung parenchyma cavities were the most frequent abnormal findings. The bronchial thickening and bronchiolar micronodules regressed after allo-HSCT, whereas bronchiectasis and residual parenchymal cavities were stable.

Conclusions: allo-HSCT seems likely to protect the long-term pulmonary prognosis of adults with IEIs; it is associated with a significantly lower incidence of severe respiratory infections, better lung function, and radiologic stabilization of lung damage.

Keywords: Inborn errors of immunity; hematopoietic stem cell transplantation; primary immunodeficiency; respiratory infections.

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Conflict of interest statement

Disclosure statement Funding: This research did not receive any specific funding from agencies or organizations in the public, commercial, or not-for-profit sector. Disclosure of potential conflict of interest: A. Benattia reports travel grants from Boehringer Ingelheim, Asten Santé, and Epione Santé, outside the submitted work. H. Salvator reports travel grants from LVL Medical-ORKYN and Oxyvie, outside the submitted work. L.-J. Couderc reports travel grants from France Oxygène, Aria Médical, and Air Liquide, outside the submitted work. A. Le Gal reports travel grants from LVL Medical-ORKYN, outside the submitted work. A. Tazi reports personal fees for lectures from Boehringer Ingelheim and travel grants from Vitalaire and Isis Medical, outside the submitted work. The rest of the authors declare that they have no relevant conflicts of interest.

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