Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up
Systemic sclerosis is an autoimmune connective tissue disease affecting both skin and internal organs. Progressive disease with multiple organ involvement is considered to have a poor prognosis. Treatment possibilities are limited, but certain patients may benefit from autologous hematopoietic stem...
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Termedia Publishing House
2019-02-01
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doaj-441549d868964bedab6e25a3e066590f2020-11-25T03:00:39ZengTermedia Publishing HouseRheumatology0034-62332084-98342019-02-01571505410.5114/reum.2019.8324035943Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-upKatarzyna Pawlak-BuśWiktor SchmidtMichał OlejarzAnna CzyżMieczysław KomarnickiPiotr LeszczyńskiSystemic sclerosis is an autoimmune connective tissue disease affecting both skin and internal organs. Progressive disease with multiple organ involvement is considered to have a poor prognosis. Treatment possibilities are limited, but certain patients may benefit from autologous hematopoietic stem cell transplantation (auto-HSCT). We report a case of a 30-year-old woman with progressive diffuse systemic sclerosis treated with parenteral cyclophosphamide with unsatisfactory results. Due to progression of the disease and lack of alternative therapies auto-HSCT was performed. After instituting treatment with autologous hematopoietic stem cell transplantation no immunosuppressive therapy has been required during 5-year follow-up. Improvement in exertion tolerance, partial regression of skin lesions and stabilization of pulmonary and cardiovascular changes were observed. Currently therapeutic options in patients with progressive systemic sclerosis are limited. Hematopoietic stem cell transplantation might become an alternative therapeutic solution not only in the early phase of the disease but also among selected patients with progressive systemic sclerosis resistant to standard therapy.https://www.termedia.pl/Autologous-hematopoietic-stem-cell-transplant-for-progressive-diffuse-systemic-sclerosis-procedural-success-and-clinical-outcome-in-5-year-follow-up,18,35943,1,1.htmlscleroderma progressive systemic sclerosis stem cell transplantation hematopoietic stem cells |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Katarzyna Pawlak-Buś Wiktor Schmidt Michał Olejarz Anna Czyż Mieczysław Komarnicki Piotr Leszczyński |
spellingShingle |
Katarzyna Pawlak-Buś Wiktor Schmidt Michał Olejarz Anna Czyż Mieczysław Komarnicki Piotr Leszczyński Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up Rheumatology scleroderma progressive systemic sclerosis stem cell transplantation hematopoietic stem cells |
author_facet |
Katarzyna Pawlak-Buś Wiktor Schmidt Michał Olejarz Anna Czyż Mieczysław Komarnicki Piotr Leszczyński |
author_sort |
Katarzyna Pawlak-Buś |
title |
Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up |
title_short |
Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up |
title_full |
Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up |
title_fullStr |
Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up |
title_full_unstemmed |
Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up |
title_sort |
autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up |
publisher |
Termedia Publishing House |
series |
Rheumatology |
issn |
0034-6233 2084-9834 |
publishDate |
2019-02-01 |
description |
Systemic sclerosis is an autoimmune connective tissue disease affecting both skin and internal organs. Progressive disease with multiple organ involvement is considered to have a poor prognosis. Treatment possibilities are limited, but certain patients may benefit from autologous hematopoietic stem cell transplantation (auto-HSCT).
We report a case of a 30-year-old woman with progressive diffuse systemic sclerosis treated with parenteral cyclophosphamide with unsatisfactory results. Due to progression of the disease and lack of alternative therapies auto-HSCT was performed.
After instituting treatment with autologous hematopoietic stem cell transplantation no immunosuppressive therapy has been required during 5-year follow-up. Improvement in exertion tolerance, partial regression of skin lesions and stabilization of pulmonary and cardiovascular changes were observed. Currently therapeutic options in patients with progressive systemic sclerosis are limited. Hematopoietic stem cell transplantation might become an alternative therapeutic solution not only in the early phase of the disease but also among selected patients with progressive systemic sclerosis resistant to standard therapy. |
topic |
scleroderma progressive systemic sclerosis stem cell transplantation hematopoietic stem cells |
url |
https://www.termedia.pl/Autologous-hematopoietic-stem-cell-transplant-for-progressive-diffuse-systemic-sclerosis-procedural-success-and-clinical-outcome-in-5-year-follow-up,18,35943,1,1.html |
work_keys_str_mv |
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