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Front Immunol ; 13: 886540, 2022.
Article in English | MEDLINE | ID: mdl-35720367

ABSTRACT

We report two patients with DNA repair disorders (Artemis deficiency, Ataxia telangiectasia) with destructive skin granulomas, presumably triggered by live-attenuated rubella vaccinations. Both patients showed reduced naïve T cells. Rapid resolution of skin lesions was observed following hematopoietic stem cell transplantation. However, the patient with AT died due to complications of severe hepatic veno-occlusive disease 6 month after HSCT. Dried blood spots obtained after birth were available from this patient and showed absent T-cell receptor excision circles (TRECs). Therefore, newborn screening may help to prevent patients with moderate T-cell deficiency from receiving live-attenuated rubella vaccine potentially causing granulomas.


Subject(s)
Ataxia Telangiectasia , DNA Repair-Deficiency Disorders , Hematopoietic Stem Cell Transplantation , Immunologic Deficiency Syndromes , Ataxia Telangiectasia/genetics , Child , DNA Repair-Deficiency Disorders/complications , Granuloma/genetics , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Immunologic Deficiency Syndromes/genetics , Infant, Newborn , Rubella virus/genetics
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