Immune Reconstitution Inflammatory Syndrome as a Posttransplantation Complication in Primary Immunodeficiency With Disseminated Mycobacterium avium

Manion, Maura; Dimitrova, Dimana; Pei, Luxin; Gea‐Banacloche, Juan; Zelazny, Adrian M.; Lisco, Andrea; Zerbe, Christa S.; Freeman, Alexandra F.; Holland, Steven M.; Kanakry, Christopher G.; Kanakry, Jennifer A.; Sereti, Irini

doi:10.1093/cid/ciz507

Cited by 12 publications

(16 citation statements)

References 15 publications

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“…The rapid improvement of the brain contrast‐enhancing lesions developed 3 months after HSCT by our patient has never been reported in the literature and may suggest an immune reconstitution inflammatory syndrome–like phenomenon, 10 whose correlation with the SAMD9L mutation requires further studies.…”

Section: Gene Loss Of Function Gain Of Functionmentioning

confidence: 53%

“…5 On the contrary, progressive leukoencephalopathy and brain atrophy have been described in patients with ataxia pancytopenia syndrome (ATXPC), MIM 159550, secondary to germline gain-of-function SAMD9L missense mutations (Table 1). 9 The rapid improvement of the brain contrast-enhancing lesions developed 3 months after HSCT by our patient has never been reported in the literature and may suggest an immune reconstitution inflammatory syndrome-like phenomenon, 10 whose correlation with the SAMD9L mutation requires further studies.…”

Section: Progression Of Non-hematologic Manifestations In Samd9lassociated Autoinflammatory Disease (Saad) After Hematopoietic Stem Cell mentioning

confidence: 61%

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Progression of non‐hematologic manifestations in SAMD9L‐associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation

Papa

Rusmini

Volpi

et al. 2021

Pediatric Allergy Immunology

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Section: Gene Loss Of Function Gain Of Functionmentioning

confidence: 53%

Section: Progression Of Non-hematologic Manifestations In Samd9lassociated Autoinflammatory Disease (Saad) After Hematopoietic Stem Cell mentioning

confidence: 61%

Progression of non‐hematologic manifestations in SAMD9L‐associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation

Papa

Rusmini

Volpi

et al. 2021

Pediatric Allergy Immunology

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“…IRIS is rare in patients after HSCT. Although IRIS was reported to be related to the discontinuation of immunosuppressive drugs (3,4), in some cases, there was no significant correlation with this (5). Most patients developed IRIS within 3 months after HSCT (6, 7), but occasionally later, up to ~2 years after transplantation (5).…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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.

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“…Postengraftment inflammatory phenomena were observed in several patients, in response to disseminated mycobacteria (n = 3) [22], Demodex (n = 2), and Aspergillus (n = 1), one of whom required systemic steroids to dampen the inflammatory immune response and one of whom had dramatic improvement upon receiving steroids for aGVHD.…”

Section: Immune Reconstitution and Phenotype Reversalmentioning

confidence: 99%

Prospective Study of a Novel, Radiation-Free, Reduced-Intensity Bone Marrow Transplantation Platform for Primary Immunodeficiency Diseases

Dimitrova

Gea‐Banacloche

Steinberg

et al. 2020

Biology of Blood and Marrow Transplantation

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Allogeneic blood or marrow transplantation (BMT) is a potentially curative therapy for patients with primary immunodeficiency (PID). Safe and effective reduced-intensity conditioning (RIC) approaches that are associated with low toxicity, use alternative donors, and afford good immune reconstitution are needed to advance the field. Twenty PID patients, ranging in age from 4 to 58 years, were treated on a prospective clinical trial of a novel, radiation-free and serotherapy-free RIC, T-cell-replete BMT approach using pentostatin, low-dose cyclophosphamide, and busulfan for conditioning with post-transplantation cyclophosphamide-based graft-versus-host-disease (GVHD) prophylaxis. This was a high-risk cohort with a median hematopoietic cell transplantation comorbidity index of 3. With median follow-up of survivors of 1.9 years, 1-year overall survival was 90% and grade III to IV acute GVHD-free, graft-failure-free survival was 80% at day +180. Graft failure incidence was 10%. Split chimerism was frequently observed at early post-BMT timepoints, with a lower percentage of donor T cells, which gradually increased by day +60. The cumulative incidences of grade II to IV and grade III to IV acute GVHD (aGVHD) were 15% and 5%, respectively. All aGVHD was steroid responsive. No patients developed chronic GVHD. Few significant organ toxicities were observed. Evidence of phenotype reversal was observed for all engrafted patients, even those with significantly mixed chimerism (n = 2) or with unknown underlying genetic defect (n = 3). All 6 patients with pre-BMT malignancies or lymphoproliferative disorders remain in remission. Most patients have discontinued immunoglobulin replacement. All survivors are off immunosuppression for GVHD prophylaxis or treatment. This novel RIC BMT approach for patients with PID has yielded promising results, even for high-risk patients. Published by Elsevier Inc. on behalf of the American Society for Transplantation and Cellular Therapy.

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Immune Reconstitution Inflammatory Syndrome as a Posttransplantation Complication in Primary Immunodeficiency With Disseminated Mycobacterium avium

Cited by 12 publications

References 15 publications

Progression of non‐hematologic manifestations in SAMD9L‐associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation

Progression of non‐hematologic manifestations in SAMD9L‐associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation

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

Prospective Study of a Novel, Radiation-Free, Reduced-Intensity Bone Marrow Transplantation Platform for Primary Immunodeficiency Diseases

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