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Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus

Vascular tumors associated with Kasabach–Merritt phenomenon (KMP) are life‐threatening and the mortality is as high as 10–30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid‐resistant cases. In the present study, analyzed data are presented to support th...

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Autores principales: Tan, Xiaoyun, Zhang, Jing, Zhou, Shaoyi, Liu, Zhenyin, Zhang, Tao, Xia, Jiejun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5947617/
https://www.ncbi.nlm.nih.gov/pubmed/29356076
http://dx.doi.org/10.1111/1346-8138.14231
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author Tan, Xiaoyun
Zhang, Jing
Zhou, Shaoyi
Liu, Zhenyin
Zhang, Tao
Xia, Jiejun
author_facet Tan, Xiaoyun
Zhang, Jing
Zhou, Shaoyi
Liu, Zhenyin
Zhang, Tao
Xia, Jiejun
author_sort Tan, Xiaoyun
collection PubMed
description Vascular tumors associated with Kasabach–Merritt phenomenon (KMP) are life‐threatening and the mortality is as high as 10–30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid‐resistant cases. In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid‐resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital. The time to initial response was 6.8 ± 2.7 days. The average stabilization time for the platelet count was 19.1 ± 8.5 days. At the time of publication, the average duration of sirolimus treatment was 14.1 ± 4.0 months, and the average time for sirolimus treatment as a single agent was 12.6 ± 4.2 months. The side‐effects were tolerable and included oral ulcer, fever, pain, skin rash and transient ascension of serum transaminase and cholesterol. Our study indicated that sirolimus therapy is an effective and safe method for the treatment of corticosteroid resistant vascular tumors associated with KMP in infants.
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spelling pubmed-59476172018-05-17 Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus Tan, Xiaoyun Zhang, Jing Zhou, Shaoyi Liu, Zhenyin Zhang, Tao Xia, Jiejun J Dermatol Concise Communications Vascular tumors associated with Kasabach–Merritt phenomenon (KMP) are life‐threatening and the mortality is as high as 10–30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid‐resistant cases. In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid‐resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital. The time to initial response was 6.8 ± 2.7 days. The average stabilization time for the platelet count was 19.1 ± 8.5 days. At the time of publication, the average duration of sirolimus treatment was 14.1 ± 4.0 months, and the average time for sirolimus treatment as a single agent was 12.6 ± 4.2 months. The side‐effects were tolerable and included oral ulcer, fever, pain, skin rash and transient ascension of serum transaminase and cholesterol. Our study indicated that sirolimus therapy is an effective and safe method for the treatment of corticosteroid resistant vascular tumors associated with KMP in infants. John Wiley and Sons Inc. 2018-01-21 2018-05 /pmc/articles/PMC5947617/ /pubmed/29356076 http://dx.doi.org/10.1111/1346-8138.14231 Text en © 2018 The Authors. The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association. This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Concise Communications
Tan, Xiaoyun
Zhang, Jing
Zhou, Shaoyi
Liu, Zhenyin
Zhang, Tao
Xia, Jiejun
Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus
title Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus
title_full Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus
title_fullStr Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus
title_full_unstemmed Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus
title_short Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus
title_sort successful management of steroid‐resistant vascular tumors associated with the kasabach–merritt phenomenon using sirolimus
topic Concise Communications
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5947617/
https://www.ncbi.nlm.nih.gov/pubmed/29356076
http://dx.doi.org/10.1111/1346-8138.14231
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