AUTHOR=Liu Shuangjun , Huo Feng , Dai Guorui , Wu Jie , Qin Maoquan , Mao Huawei , Wang Quan TITLE=Case Report: Immune reconstitution inflammatory syndrome after hematopoietic stem cell transplantation for severe combined immunodeficiency JOURNAL=Frontiers in Immunology VOLUME=13 YEAR=2022 URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2022.960749 DOI=10.3389/fimmu.2022.960749 ISSN=1664-3224 ABSTRACT=

We report a case of immune reconstitution inflammatory syndrome (IRIS) after hematopoietic stem cell transplantation (HSCT). The patient had sever bacillus Calmette–Guerin (BCG) vaccine–caused disseminated infection and had received allogeneic HSCT for X-linked severe combined immunodeficiency disease. After HSCT, complicated by treatment-responding veno-occlusive disease and acute graft-versus-host disease, at the time when immunosuppressants were withdrawn, the patient experienced recurrent fever accompanied by elevated inflammatory indicators. After receiving glucocorticoids and ibuprofen, the patient’s condition improved, and a diagnosis with BCG-related IRIS was made.