Cargando…

SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report

Graft-vs. -host disease (GvHD) is a serious and complex immunological complication of haematopoietic stem cell transplantation (HSCT) and is associated with prolonged immunodeficiency and non-relapse mortality. Standard treatment of chronic GvHD comprises steroids in combination with other immunosup...

Descripción completa

Detalles Bibliográficos
Autores principales: Zubarovskaya, Natalia, Hofer-Popow, Irene, Idzko, Marco, Haas, Oskar A., Lawitschka, Anita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8795895/
https://www.ncbi.nlm.nih.gov/pubmed/35096703
http://dx.doi.org/10.3389/fped.2021.775318
_version_ 1784641177767641088
author Zubarovskaya, Natalia
Hofer-Popow, Irene
Idzko, Marco
Haas, Oskar A.
Lawitschka, Anita
author_facet Zubarovskaya, Natalia
Hofer-Popow, Irene
Idzko, Marco
Haas, Oskar A.
Lawitschka, Anita
author_sort Zubarovskaya, Natalia
collection PubMed
description Graft-vs. -host disease (GvHD) is a serious and complex immunological complication of haematopoietic stem cell transplantation (HSCT) and is associated with prolonged immunodeficiency and non-relapse mortality. Standard treatment of chronic GvHD comprises steroids in combination with other immunosuppressive agents. Extracorporeal photopheresis (ECP), with its immunomodulatory mechanism, is applied as part of steroid-sparing regimens for chronic GvHD. Immunocompromised, chronically ill patients are at particular risk of severe disease courses of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. T-cell immunity in SARS-CoV-2 infection is well-described but the role of the humoral immune responses is not fully understood. This case report describes a moderate course of SARS-CoV-2 infection in a patient <9 months after HSCT who was suffering from active, severe, chronic GvHD treated with prednisone and ECP. Following HSCT from a matched unrelated donor to cure acute lymphoblastic leukaemia, the 25-year-old male patient experienced multiple infectious complications associated with cytopenia, B-cell dyshomeostasis and autoantibody production followed by development of severe chronic GvHD thereafter at day +212. The steroid-sparing treatment plan consisted of supportive care, topical treatment, prednisone and ECP. He was diagnosed with SARS-CoV-2 infection at day +252, experiencing loss of smell and taste as well as a cough. The patient's oxygen saturation was between 94 and 97% on room air, and computed tomography images showed evolution of typical of SARS-CoV-2 infiltrates. In addition to cytopenia and immune dyshomeostasis, laboratory tests confirmed macrophage activating syndrome, transaminitis and Epstein-Barr virus viraemia. At that time, anti-SARS-CoV-2 monoclonal antibodies were not available in Austria and remdesivir seemed contraindicated. Surprisingly, despite severe lymphopenia the patient developed SARS-CoV-2-specific antibodies within 15 days, which was followed by clearance of SARS-CoV-2 and EBV with resolution of symptoms. Thereafter, parameters of immune dysregulation such as lymphopenia and B-cell dyshomeostasis, the latter characterised by elevated CD21(low) B cells and autoantibody expression, normalised. Moreover, we observed complete response of active chronic GvHD to treatment.
format Online
Article
Text
id pubmed-8795895
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-87958952022-01-29 SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report Zubarovskaya, Natalia Hofer-Popow, Irene Idzko, Marco Haas, Oskar A. Lawitschka, Anita Front Pediatr Pediatrics Graft-vs. -host disease (GvHD) is a serious and complex immunological complication of haematopoietic stem cell transplantation (HSCT) and is associated with prolonged immunodeficiency and non-relapse mortality. Standard treatment of chronic GvHD comprises steroids in combination with other immunosuppressive agents. Extracorporeal photopheresis (ECP), with its immunomodulatory mechanism, is applied as part of steroid-sparing regimens for chronic GvHD. Immunocompromised, chronically ill patients are at particular risk of severe disease courses of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. T-cell immunity in SARS-CoV-2 infection is well-described but the role of the humoral immune responses is not fully understood. This case report describes a moderate course of SARS-CoV-2 infection in a patient <9 months after HSCT who was suffering from active, severe, chronic GvHD treated with prednisone and ECP. Following HSCT from a matched unrelated donor to cure acute lymphoblastic leukaemia, the 25-year-old male patient experienced multiple infectious complications associated with cytopenia, B-cell dyshomeostasis and autoantibody production followed by development of severe chronic GvHD thereafter at day +212. The steroid-sparing treatment plan consisted of supportive care, topical treatment, prednisone and ECP. He was diagnosed with SARS-CoV-2 infection at day +252, experiencing loss of smell and taste as well as a cough. The patient's oxygen saturation was between 94 and 97% on room air, and computed tomography images showed evolution of typical of SARS-CoV-2 infiltrates. In addition to cytopenia and immune dyshomeostasis, laboratory tests confirmed macrophage activating syndrome, transaminitis and Epstein-Barr virus viraemia. At that time, anti-SARS-CoV-2 monoclonal antibodies were not available in Austria and remdesivir seemed contraindicated. Surprisingly, despite severe lymphopenia the patient developed SARS-CoV-2-specific antibodies within 15 days, which was followed by clearance of SARS-CoV-2 and EBV with resolution of symptoms. Thereafter, parameters of immune dysregulation such as lymphopenia and B-cell dyshomeostasis, the latter characterised by elevated CD21(low) B cells and autoantibody expression, normalised. Moreover, we observed complete response of active chronic GvHD to treatment. Frontiers Media S.A. 2022-01-14 /pmc/articles/PMC8795895/ /pubmed/35096703 http://dx.doi.org/10.3389/fped.2021.775318 Text en Copyright © 2022 Zubarovskaya, Hofer-Popow, Idzko, Haas and Lawitschka. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Zubarovskaya, Natalia
Hofer-Popow, Irene
Idzko, Marco
Haas, Oskar A.
Lawitschka, Anita
SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report
title SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report
title_full SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report
title_fullStr SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report
title_full_unstemmed SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report
title_short SARS-CoV-2 Infection and Active, Multiorgan, Severe cGVHD After HSCT for Adolescent ALL: More Luck Than Understanding? A Case Report
title_sort sars-cov-2 infection and active, multiorgan, severe cgvhd after hsct for adolescent all: more luck than understanding? a case report
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8795895/
https://www.ncbi.nlm.nih.gov/pubmed/35096703
http://dx.doi.org/10.3389/fped.2021.775318
work_keys_str_mv AT zubarovskayanatalia sarscov2infectionandactivemultiorganseverecgvhdafterhsctforadolescentallmoreluckthanunderstandingacasereport
AT hoferpopowirene sarscov2infectionandactivemultiorganseverecgvhdafterhsctforadolescentallmoreluckthanunderstandingacasereport
AT idzkomarco sarscov2infectionandactivemultiorganseverecgvhdafterhsctforadolescentallmoreluckthanunderstandingacasereport
AT haasoskara sarscov2infectionandactivemultiorganseverecgvhdafterhsctforadolescentallmoreluckthanunderstandingacasereport
AT lawitschkaanita sarscov2infectionandactivemultiorganseverecgvhdafterhsctforadolescentallmoreluckthanunderstandingacasereport