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

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

2022Frontiers in Immunology13 citationsDOIOpen Access PDF

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veno-occlusive disease and acute graft-versus-host disease, at the time when immunosuppressants were withdrawn, the patient experienced recurrent fever accompanied by elevated inflammatory indicators.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

Topics & Concepts

MedicineHematopoietic stem cell transplantationImmune reconstitution inflammatory syndromePrimary immunodeficiencyImmunodeficiencyImmunologySevere combined immunodeficiencyTransplantationDiseaseImmune systemStem cellGraft-versus-host diseaseInternal medicineHuman immunodeficiency virus (HIV)BiologyViral loadAntiretroviral therapyGeneGeneticsBiochemistryInfectious Diseases and TuberculosisBlood disorders and treatmentsPneumocystis jirovecii pneumonia detection and treatment