Author:
Yamashita Motoi,Eguchi Shiori,Tomomasa Dan,Kamiya Takahiro,Niizato Daiki,Mitsuiki Noriko,Isoda Takeshi,Funakoshi Hanako,Mizuno Yuki,Okamoto Kentaro,Nguyen Tuan Minh,Takada Hidetoshi,Takagi Masatoshi,Imai Kohsuke,Morio Tomohiro,Kanegane Hirokazu
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 graft-versus-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 veno-occlusive 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.
Funder
Japan Society for the Promotion of Science
Subject
Immunology,Immunology and Allergy
Cited by
2 articles.
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