Successful treatment of BCG‐related immune reconstitution inflammatory syndrome following ex vivo T‐cell–depleted haploidentical hematopoietic stem cell transplantation: A case report

Abstract

IRIS is a phenomenon describing localized inflammatory reactions at BCG vaccination site and development of lymphadenopathy as immune system recovers. It is a rare entity in children following haploidentical HSCT. We represent the successful treatment of a case with fluctuating lymphadenopathy due to BCG vaccine during immune reconstitution period following ex vivo T‐cell–depleted haploidentical HSCT.

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

“…Not all IRIS requires treatment, and the majority of patients with IRIS have a good prognosis (5,(11)(12)(13)(14). In addition to glucocorticoids and immunosuppressants, other treatment for IRIS includes non-steroidal anti-inflammatory drugs (NSAIDs) and immunosuppressants (11,15,16).…”

“…Unmasking IRIS refers to the worsening of a pre-existing infection that has not been detected during the process of immune recovery, whereas paradoxical IRIS refers to the worsening of a confirmed pre-existing infection. Nine cases of paradoxical IRIS after HSCT were reported, and Mycobacterium was the most common pathogen ( 3 , 4 , 11 , 12 ). We speculate that the IRIS of our patient was related to immune dysregulation following successful HSCT, including pre-existing BCG infection, immune reconstitution after HSCT, and withdrawal of immunosuppressants.…”

Bacillus Calmette–Guerin (BCG) Vaccine-associated Complications in Immunodeficient Patients Following Stem Cell Transplantation

“…There are currently no guidelines for treatment of post-HSCT BCG disease, and diverse regimens have been used [ 3 , 18 – 21 , 24 – 28 , 31 , 33 , 35 , 39 , 48 , 49 ]. Treatment modalities other than anti-microbials include steroids [ 27 , 29 , 44 , 47 , 48 ], surgical treatment, [ 19 , 20 , 23 , 27 , 48 ] IFNγ, [ 18 ] and anti-IL1 and anti-IL-6R antagonists [ 27 ]. Our treatment protocol includes a triple regimen until clinical and radiological resolution of disease, as well as immune reconstitution.…”