Haploidentical allogeneic hematopoietic stem cell transplantation in treatment of chronic granulomatous disease with chronic active Epstein-Barr virus infection: report of one case and review of literature / 白血病·淋巴瘤

ABSTRACT

Objective:To analyze the clinical characteristics of chronic granulomatous disease (CGD) with chronic active EB virus (EBV) infection (CAEBV).Methods:The clinical data of 1 CGD patient combined with CAEBV who underwent allogeneic hematopoietic stem cell transplantation (HSCT) in Beijing Friendship Hospital of Capital Medical University in March 2017 was retrospectively analyzed, and the relevant literature was also summarized.Results:After transplantation, the chimeric rate and gene test was 100% complete donor type. During the follow-up period, no recurrent severe infection occurred within 39 months, and EBV-DNA copy was 0; no acute or chronic graft-versus-host disease (GVHD) related manifestations occurred, and the quality of life was higher.Conclusions:Haploid allogeneic HSCT with myeloablative conditioning regimens is a relatively safe and effective treatment for CGD patients combined with CAEBV. For primary immunodeficiency disease, it suggests that the haploidentical donor carrying the monohybrid pathogenic gene can also be used as an effective stem cell source if the hematological and immunological function is normal.