Case Report: Immune reconstitution inflammatory syndrome after hematopoietic stem cell transplantation for severe combined immunodeficiency

Shuangjun Liu; Feng Huo; Guorui Dai; Jie Wu; Maoquan Qin; Huawei Mao; Quan Wang

doi:10.3389/fimmu.2022.960749

Case Report: Immune reconstitution inflammatory syndrome after hematopoietic stem cell transplantation for severe combined immunodeficiency

Front Immunol. 2022 Sep 26:13:960749. doi: 10.3389/fimmu.2022.960749. eCollection 2022.

Authors

Shuangjun Liu¹, Feng Huo¹, Guorui Dai¹, Jie Wu¹, Maoquan Qin¹, Huawei Mao¹, Quan Wang¹

Affiliation

¹ Beijing Children's Hospital, Capital Medical University, Beijing, China.

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veno-occlusive disease and acute graft-versus-host disease, at the time when immunosuppressants were withdrawn, the patient experienced recurrent fever accompanied by elevated inflammatory indicators.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.

Keywords: BCG disseminated tuberculosis; HSCT = hematopoietic stem cell transplant; IL2RG; IRIS - immune reconstitution inflammatory syndrome; severe combined immunodeficiency.

Publication types

Case Reports

MeSH terms

BCG Vaccine / adverse effects
Hematopoietic Stem Cell Transplantation* / adverse effects
Humans
Ibuprofen
Immune Reconstitution Inflammatory Syndrome* / diagnosis
Immune Reconstitution Inflammatory Syndrome* / etiology
Immunosuppressive Agents
Severe Combined Immunodeficiency*

Substances

BCG Vaccine
Immunosuppressive Agents
Ibuprofen