Successful second bone marrow transplantation in Omenn’s syndrome after bone marrow aplasia: A case report

Wahadneh, Adel; Bin-Dahman, Haifa; Habahbeh, Zeyad; Abu-Shukear, Mohammad; Ajarmeh, Mohammad A; Zyood, Raed; Khaled, Ahmad

doi:10.1111/j.1399-3046.2010.01413.x

Cited by 4 publications

(2 citation statements)

References 16 publications

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“…Intermittent fever, generalized lymphadenopathies, hepatosplenomegaly, cough, bone involvement, weight loss and skin rash may develop later in the course of the disease [15,26]. Refractory cough due to recurrent lower respiratory infections is another presentation of the disease reported in several cases [28], without significant changes in chest radiography [4]. Osseous involvement is another finding in disseminated BCG infection [6].…”

Section: Discussionmentioning

confidence: 99%

Disseminated Bacille Calmette-Guérin Infection at a Glance: A Mini Review of the Literature

Hassanzad

Valinejadi

Darougar

et al. 2019

Advances in Respiratory Medicine

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Introduction: Immunodeficient children are at a high risk of disseminated Bacillus Calmette-Guérin [BCG] infection. We assessed the literature on clinical manifestations of BCGosis in children with specific primary immunodeficiencies. Material and methods: We conducted a systematic review of clinical practice articles by searching Medline, PubMed, Embase, Scopus, Web of Science and Google Scholar from their inception to date. Results: Thirty-seven articles were included regarding BCG vaccination and its dissemination in children with primary immunodeficiencies. Articles on dissemination after intravesicular BCG were excluded from the study. Conclusions: Since disseminated BCG vaccination may be the first manifestation of a primary immunodeficiency disease, a comprehensive search for immunological defects in children developing these problems after BCG vaccination seems rational.

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Section: Discussionmentioning

confidence: 99%

Disseminated Bacille Calmette-Guérin Infection at a Glance: A Mini Review of the Literature

Hassanzad

Valinejadi

Darougar

et al. 2019

Advances in Respiratory Medicine

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“…Omenn syndrome (OS) is one of the form of severe combined immunodeficiency (SCID) with autoreactive manifestations. Due to immune dysregulation, patients with OS typically have erythroderma, hepatosplenomegaly, lymphadenopathy, recurrent infections, and alopecia 1,2 .…”

Section: Introductionmentioning

confidence: 99%

Successful hematopoietic stem cell transplantation with reduced dose of busulfan for Omenn syndrome

Matsukawa

Isshiki

Osumi

et al. 2022

BCT

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Omenn syndrome (OS) is typically observed in the autosomal recessive form of severe combined immunodeficiency (SCID) with autoreactive manifestations, and it requires allogeneic hematopoietic stem cell transplantation. Unlike non-OS SCID, a conditioning regimen is usually required to eradicate T-cells; however, optimal conditioning regimens are not established mainly because of the rarity of OS. Here, we report a case of hematopoietic stem cell transplantation with a reduced dose of busulfan, as a conditioning regimen and successful engraftment with complete chimerism. OS was diagnosed in a one-month-old boy based on a diffuse erythematous rash, absent B-cells, and activated T-cells. Genetic analysis failed to identify causative mutations for OS/ SCID, such as RAG1/2. Bone marrow transplantation was performed from his HLA-matched sister with a conditioning regimen consisting of targeted busulfan, fludarabine, and anti-thymocyte globulin. Cyclosporine had been administered before transplantation to control abnormal T-cell activation and continued for graft-versushost disease (GVHD) prophylaxis. Engraftment was achieved on day 12, and no GVHD symptoms were observed. For stem cell transplantation for OS, prior control of autoreactive symptoms with immunosuppressants is important for safe transplantation and reduced intensity conditioning (RIC) can be an option to achieve sustained engraftment.

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

et al. 2020

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Purpose Bacillus Calmette–Guerin (BCG) is a live attenuated vaccine with the potential of causing severe iatrogenic complications in patients with primary immunodeficiency diseases (PID) before and after hematopoietic stem cell transplantation (HSCT). We aim to investigate risk factors of post-HSCT BCG-related complications in PID patients. Methods A retrospective analysis of pediatric PID patients who had received the BCG vaccine and underwent HSCT at Hadassah-Hebrew University Medical Center, between 2007 and 2019. Results We found 15/36 (41.67%) patients who developed post-HSCT BCG-related complications. The most significant risk factor for developing BCG-related complications was T cell deficiency (47.6% of the non-complicated vs 83.3% of the BCGitis and 100% of the BCGosis groups had T cell lymphopenia, p = 0.013). None of the chronic granulomatous patients developed BCG-related manifestation post-transplant. Among T cell–deficient patients, lower NK (127 vs 698 cells/μl, p = 0.04) cell counts and NK-SCID were risk factors for ongoing post-HSCT BCGosis, as was pretransplant disseminated BCGosis (33.3% of patients with BCGosis vs none of the non-BCGosis patients, p = 0.04). Immune reconstitution inflammatory syndrome (IRIS) was observed in 3/5 patients with Omenn syndrome. Prophylactic antimycobacterial treatment was not proven effective. Conclusion BCG vaccination can cause significant morbidity and mortality in the post-transplant T cell–deficient patient, especially in the presence of pre-transplant disease. Taking a detailed medical history prior to administering, the BCG vaccine is crucial for prevention of this complication.

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Successful second bone marrow transplantation in Omenn’s syndrome after bone marrow aplasia: A case report

Cited by 4 publications

References 16 publications

Disseminated Bacille Calmette-Guérin Infection at a Glance: A Mini Review of the Literature

Disseminated Bacille Calmette-Guérin Infection at a Glance: A Mini Review of the Literature

Successful hematopoietic stem cell transplantation with reduced dose of busulfan for Omenn syndrome

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

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