Cargando…

Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at...

Descripción completa

Detalles Bibliográficos
Autores principales: Grześk, Elżbieta, Kołtan, Sylwia, Dąbrowska, Anna, Urbańczyk, Anna, Małdyk, Jadwiga, Małkowski, Bogdan, Bogiel, Tomasz, Dębski, Robert, Czyżewski, Krzysztof, Wysocki, Mariusz, Styczyński, Jan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9390486/
https://www.ncbi.nlm.nih.gov/pubmed/35990691
http://dx.doi.org/10.3389/fimmu.2022.915986
_version_ 1784770665648226304
author Grześk, Elżbieta
Kołtan, Sylwia
Dąbrowska, Anna
Urbańczyk, Anna
Małdyk, Jadwiga
Małkowski, Bogdan
Bogiel, Tomasz
Dębski, Robert
Czyżewski, Krzysztof
Wysocki, Mariusz
Styczyński, Jan
author_facet Grześk, Elżbieta
Kołtan, Sylwia
Dąbrowska, Anna
Urbańczyk, Anna
Małdyk, Jadwiga
Małkowski, Bogdan
Bogiel, Tomasz
Dębski, Robert
Czyżewski, Krzysztof
Wysocki, Mariusz
Styczyński, Jan
author_sort Grześk, Elżbieta
collection PubMed
description Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infections especially viral and malignancies than the general population. The aim of the study was to present complex diagnostic and therapeutic management of HV-LPD. The clinical diagnosis was confirmed at the histological and molecular level with next generation sequencing. HV-LPD was diagnosed in a patient with CVID and chronic active Epstein–Barr virus (CAEBV) infection. The patient was refractory to CHOP chemotherapy and immunosuppressive treatment in combination with antiviral drugs (prednisone, bortezomib, gancyclovir). The third-party donor EBV-specific cytotoxic T cells (EBV-CTL, tabelecleucel) were used, which stabilised the disease course. Finally, matched unrelated donor hematopoietic cell transplantation (MUD-HCT) was performed followed by another cycle of EBV-CTL.
format Online
Article
Text
id pubmed-9390486
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-93904862022-08-20 Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection Grześk, Elżbieta Kołtan, Sylwia Dąbrowska, Anna Urbańczyk, Anna Małdyk, Jadwiga Małkowski, Bogdan Bogiel, Tomasz Dębski, Robert Czyżewski, Krzysztof Wysocki, Mariusz Styczyński, Jan Front Immunol Immunology Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infections especially viral and malignancies than the general population. The aim of the study was to present complex diagnostic and therapeutic management of HV-LPD. The clinical diagnosis was confirmed at the histological and molecular level with next generation sequencing. HV-LPD was diagnosed in a patient with CVID and chronic active Epstein–Barr virus (CAEBV) infection. The patient was refractory to CHOP chemotherapy and immunosuppressive treatment in combination with antiviral drugs (prednisone, bortezomib, gancyclovir). The third-party donor EBV-specific cytotoxic T cells (EBV-CTL, tabelecleucel) were used, which stabilised the disease course. Finally, matched unrelated donor hematopoietic cell transplantation (MUD-HCT) was performed followed by another cycle of EBV-CTL. Frontiers Media S.A. 2022-08-05 /pmc/articles/PMC9390486/ /pubmed/35990691 http://dx.doi.org/10.3389/fimmu.2022.915986 Text en Copyright © 2022 Grześk, Kołtan, Dąbrowska, Urbańczyk, Małdyk, Małkowski, Bogiel, Dębski, Czyżewski, Wysocki and Styczyński 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 Immunology
Grześk, Elżbieta
Kołtan, Sylwia
Dąbrowska, Anna
Urbańczyk, Anna
Małdyk, Jadwiga
Małkowski, Bogdan
Bogiel, Tomasz
Dębski, Robert
Czyżewski, Krzysztof
Wysocki, Mariusz
Styczyński, Jan
Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection
title Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection
title_full Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection
title_fullStr Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection
title_full_unstemmed Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection
title_short Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection
title_sort case report: cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active epstein-barr virus infection
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9390486/
https://www.ncbi.nlm.nih.gov/pubmed/35990691
http://dx.doi.org/10.3389/fimmu.2022.915986
work_keys_str_mv AT grzeskelzbieta casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT kołtansylwia casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT dabrowskaanna casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT urbanczykanna casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT małdykjadwiga casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT małkowskibogdan casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT bogieltomasz casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT debskirobert casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT czyzewskikrzysztof casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT wysockimariusz casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection
AT styczynskijan casereportcellulartherapyforhydroavacciniformelikelymphoproliferativedisorderinpediatriccommonvariableimmunodeficiencywithchronicactiveepsteinbarrvirusinfection