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CASE REPORT article

Front. Immunol.
Sec. Primary Immunodeficiencies
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1475448

Dual In Vivo T Cell Depleted Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant Cyclophosphamide and Anti-Thymocyte Globulin as a Third Salvage Transplant for Leukocyte Adhesion Deficiency with Graft Failure: a case report

Provisionally accepted
Azadeh Kiumarsi Azadeh Kiumarsi 1Amirarsalan Alaei Amirarsalan Alaei 2Mohsen Nikbakht Mohsen Nikbakht 3Saeed Mohammadi Saeed Mohammadi 3*Mohammad Reza Ostad-Ali Mohammad Reza Ostad-Ali 3*Zahra Rasti Zahra Rasti 3*Tahereh Rostami Tahereh Rostami 3*
  • 1 Department of Pediatrics, School of Medicine, Childrens Medical Center, Tehran University of Medical Sciences, Tehran, Iran., Tehran, Alborz, Iran
  • 2 Immunology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
  • 3 Tehran University of Medical Sciences, Tehran, Tehran, Iran

The final, formatted version of the article will be published soon.

    Background: With recent advances in clinical practice, including the use of reduced-toxicity conditioning regimens and innovative approaches such as ex vivo TCRαβ/CD19 depletion of haploidentical donor stem cells or post-transplant cyclophosphamide (PTCY), hematopoietic stem cell transplantation (HSCT) has emerged as a curative treatment option for a growing population of patients with inborn errors of immunity (IEI). However, despite these promising developments, graft failure (GF) remains a significant concern associated with HSCT in these patients. Although a second HSCT is the only established salvage therapy for patients who experience GF, there are no uniform, standardized strategies for performing these second transplants. Furthermore, even less data is available regarding the outcomes and best practices for a third HSCT as a salvage measure when a second HSCT fails to achieve engraftment.experienced GF after the first and second HSCT from a matched unrelated donor. As a salvage measure, the patient received a dual in vivo T-cell depleted haploidentical HSCT. The conditioning regimen for this third HSCT included anti-thymocyte globulin (ATG) and PTCY.Complete donor chimerism was assessed using the short tandem repeat (STR) PCR technique.By day +28 after the transplant, the expression of the leukocyte adhesion molecules CD18, CD11b, and CD11c on the patient's peripheral blood neutrophils had recovered to over 99%. It remained stable throughout the 18-month follow-up period.T-cell replete haploidentical HSCT with ATG and PTCY may be a viable salvage option for LAD patients who have rejected prior HSCT.

    Keywords: inborn errors of immunity (IEI), Leukocyte adhesion deficiency (LAD), Hematopoietic Stem Cell Transplantation (HSCT), haploidentical, Graft failure (GF)

    Received: 03 Aug 2024; Accepted: 16 Dec 2024.

    Copyright: © 2024 Kiumarsi, Alaei, Nikbakht, Mohammadi, Ostad-Ali, Rasti and Rostami. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Saeed Mohammadi, Tehran University of Medical Sciences, Tehran, 1417863181, Tehran, Iran
    Mohammad Reza Ostad-Ali, Tehran University of Medical Sciences, Tehran, 1417863181, Tehran, Iran
    Zahra Rasti, Tehran University of Medical Sciences, Tehran, 1417863181, Tehran, Iran
    Tahereh Rostami, Tehran University of Medical Sciences, Tehran, 1417863181, Tehran, Iran

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.