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- Title
Case report: HLA-haploidentical hematopoietic cell transplant with posttransplant cyclophosphamide in a patient with leukocyte adhesion deficiency type I.
- Authors
Motoi Yamashita; Shiori Eguchi; Dan Tomomasa; Takahiro Kamiya; Daiki Niizato; Noriko Mitsuiki; Takeshi Isoda; Hanako Funakoshi; Yuki Mizuno; Kentaro Okamoto; Tuan Minh Nguyen; Hidetoshi Takada; Masatoshi Takagi; Kohsuke Imai; Tomohiro Morio; Hirokazu Kanegane
- Abstract
Leukocyte adhesion deficiency type I (LAD-I) is a rare autosomal recessive inborn error of immunity (IEI) caused by the defects in CD18, encoded by the ITGB2 gene. LAD-I is characterized by defective leukocyte adhesion to the vascular endothelium and impaired migration of leukocytes. Allogeneic hematopoietic cell transplant (HCT) is the only curative treatment for LAD-I. In an absence of ideal donor for HCT, human leukocyte antigen (HLA)-haploidentical HCT is performed. Posttransplant cyclophosphamide (PT-CY) is a relatively new graftversus-host disease (GVHD) prophylactic measure and has been increasingly used in HLA-haploidentical HCT for malignant and nonmalignant diseases. However, experience in using PT-CY for rare IEIs, such as LAD-I, is very limited. We report a case of LAD-I successfully treated with HLA-haploidentical HCT with PT-CY. Complete chimerism was achieved, and the patient was cured. Her transplant course was complicated by mild GVHD, cytomegalovirus reactivation and venoocclusive disease/sinusoidal obstruction syndrome, which were successfully treated. HLA-haploidentical HCT with PT-CY is a safe and effective option for patients with LAD-I when HLA-matched donors are unavailable.
- Subjects
HEPATIC veno-occlusive disease; LEUCOCYTES; HLA histocompatibility antigens; CYCLOPHOSPHAMIDE; VASCULAR endothelium; TRANSPLANTATION of organs, tissues, etc.
- Publication
Frontiers in Immunology, 2022, Vol 13, p1
- ISSN
1664-3224
- Publication type
Article
- DOI
10.3389/fimmu.2022.1020362