Frontiers in Immunology (Jun 2022)
Case Report: Rubella Virus-Induced Cutaneous Granulomas in Two Pediatric Patients With DNA Double Strand Breakage Repair Disorders – Outcome After Hematopoietic Stem Cell Transplantation
- Ulrich Baumann,
- Johannes H. Schulte,
- Jonathan P. Groß,
- Rita Beier,
- Marius Ludwig,
- Volker Wahn,
- Jörg Hofmann,
- Jörg Hofmann,
- Britta Maecker-Kolhoff,
- Martin Sauer,
- Petra Kaiser-Labusch,
- Negin Karimian,
- Ulrike Blume-Peytavi,
- Franziska Ghoreschi,
- Hagen Ott,
- Ludmila Perelygina,
- Christian Klemann,
- Oliver Blankenstein,
- Horst von Bernuth,
- Horst von Bernuth,
- Horst von Bernuth,
- Horst von Bernuth,
- Renate Krüger
Affiliations
- Ulrich Baumann
- Paediatric Pulmonology, Allergy and Neonatology, Hannover Medical School, Hannover, Germany
- Johannes H. Schulte
- Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Jonathan P. Groß
- Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Rita Beier
- Paediatric Oncology and Hematology, Hannover Medical School, Hannover, Germany
- Marius Ludwig
- Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Volker Wahn
- Department of Pediatric Respiratory Medicine, Immunology and Intensive Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Jörg Hofmann
- Labor Berlin GmbH, Department of Virology, Berlin, Germany
- Jörg Hofmann
- Institute of Virology, Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
- Britta Maecker-Kolhoff
- Paediatric Oncology and Hematology, Hannover Medical School, Hannover, Germany
- Martin Sauer
- Paediatric Oncology and Hematology, Hannover Medical School, Hannover, Germany
- Petra Kaiser-Labusch
- Prof. Hess Children’s Hospital, Klinikum Bremen-Mitte, Bremen, Germany
- Negin Karimian
- Department of Dermatology and Allergology, Charité-Universitätsmedizin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Ulrike Blume-Peytavi
- Department of Dermatology and Allergology, Charité-Universitätsmedizin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Franziska Ghoreschi
- Department of Dermatology and Allergology, Charité-Universitätsmedizin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Hagen Ott
- Department of Paediatric Dermatology and Allergology, Center for Rare Congenital Skin Diseases, Children’s Hospital Auf der Bult, Hannover, Germany
- Ludmila Perelygina
- 0Division of Viral Diseases, Centers for Disease Control and Prevention, Atlanta, GA, United States
- Christian Klemann
- Paediatric Pulmonology, Allergy and Neonatology, Hannover Medical School, Hannover, Germany
- Oliver Blankenstein
- 1Institute for Experimental Pediatric Endocrinology, Newborn Screening Laboratory, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Berlin, Germany
- Horst von Bernuth
- Department of Pediatric Respiratory Medicine, Immunology and Intensive Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- Horst von Bernuth
- 2Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany
- Horst von Bernuth
- 3Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin-Brandenburg Center for Regenerative Therapies (BCRT), Berlin, Germany
- Horst von Bernuth
- 4Labor Berlin GmbH, Department of Immunology, Berlin, Germany
- Renate Krüger
- Department of Pediatric Respiratory Medicine, Immunology and Intensive Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany
- DOI
- https://doi.org/10.3389/fimmu.2022.886540
- Journal volume & issue
-
Vol. 13
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.
Keywords
- inborn errors of immunity
- primary immunodeficiency
- Ataxia telangiectasia
- Artemis deficiency
- rubella virus vaccine strain
- granuloma formation
