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Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management
PURPOSE: Musculoskeletal complications have been associated with kaposiform hemangioendothelioma (KHE) and can lead to disability and reduced quality of life. We aimed to determine the clinical characteristics of musculoskeletal complication in patients with KHE without Kasabach–Merritt phenomenon (...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dove Medical Press
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6135070/ https://www.ncbi.nlm.nih.gov/pubmed/30233248 http://dx.doi.org/10.2147/CMAR.S171223 |
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author | Ji, Yi Yang, Kaiying Chen, Siyuan Peng, Suhua Lu, Guoyan Liu, Xingtao |
author_facet | Ji, Yi Yang, Kaiying Chen, Siyuan Peng, Suhua Lu, Guoyan Liu, Xingtao |
author_sort | Ji, Yi |
collection | PubMed |
description | PURPOSE: Musculoskeletal complications have been associated with kaposiform hemangioendothelioma (KHE) and can lead to disability and reduced quality of life. We aimed to determine the clinical characteristics of musculoskeletal complication in patients with KHE without Kasabach–Merritt phenomenon (KMP) in order to identify features that may aid clinicians in KHE treatment. PATIENTS AND METHODS: We conducted a cohort study of KHE without KMP associated with musculoskeletal complication between January 2006 and February 2017 at three tertiary medical centers in China. The study included 29 nonthrombocytopenic patients with KHE and musculoskeletal complication. RESULTS: The mean age at diagnosis of KHE was 4.5 years (range, 0.3–50.0 years). The mean follow-up was 4.1 years (range, 0.5–9.0 years). In most cases (72.4%), decreased range of motion (ROM) appeared within 2 years of KHE onset. Associated chronic pain was reported in 12 patients. Bone–joint changes were common in patients with decreased ROM (75.9%). All the patients received at least one medical therapy including corticosteroids, vincristine, propranolol, and sirolimus. Sirolimus demonstrated the highest efficacy rate, with 94.7% of patients showing improvements in ROM and chronic pain. CONCLUSION: Musculoskeletal complication can occur early in the disease course of KHE without KMP. Although no uniformly effective treatment modality was found, sirolimus demonstrated the best response in patients with KHE with decreased ROM and chronic pain. |
format | Online Article Text |
id | pubmed-6135070 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Dove Medical Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-61350702018-09-19 Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management Ji, Yi Yang, Kaiying Chen, Siyuan Peng, Suhua Lu, Guoyan Liu, Xingtao Cancer Manag Res Original Research PURPOSE: Musculoskeletal complications have been associated with kaposiform hemangioendothelioma (KHE) and can lead to disability and reduced quality of life. We aimed to determine the clinical characteristics of musculoskeletal complication in patients with KHE without Kasabach–Merritt phenomenon (KMP) in order to identify features that may aid clinicians in KHE treatment. PATIENTS AND METHODS: We conducted a cohort study of KHE without KMP associated with musculoskeletal complication between January 2006 and February 2017 at three tertiary medical centers in China. The study included 29 nonthrombocytopenic patients with KHE and musculoskeletal complication. RESULTS: The mean age at diagnosis of KHE was 4.5 years (range, 0.3–50.0 years). The mean follow-up was 4.1 years (range, 0.5–9.0 years). In most cases (72.4%), decreased range of motion (ROM) appeared within 2 years of KHE onset. Associated chronic pain was reported in 12 patients. Bone–joint changes were common in patients with decreased ROM (75.9%). All the patients received at least one medical therapy including corticosteroids, vincristine, propranolol, and sirolimus. Sirolimus demonstrated the highest efficacy rate, with 94.7% of patients showing improvements in ROM and chronic pain. CONCLUSION: Musculoskeletal complication can occur early in the disease course of KHE without KMP. Although no uniformly effective treatment modality was found, sirolimus demonstrated the best response in patients with KHE with decreased ROM and chronic pain. Dove Medical Press 2018-09-07 /pmc/articles/PMC6135070/ /pubmed/30233248 http://dx.doi.org/10.2147/CMAR.S171223 Text en © 2018 Ji et al. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. |
spellingShingle | Original Research Ji, Yi Yang, Kaiying Chen, Siyuan Peng, Suhua Lu, Guoyan Liu, Xingtao Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management |
title | Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management |
title_full | Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management |
title_fullStr | Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management |
title_full_unstemmed | Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management |
title_short | Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management |
title_sort | musculoskeletal complication in kaposiform hemangioendothelioma without kasabach–merritt phenomenon: clinical characteristics and management |
topic | Original Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6135070/ https://www.ncbi.nlm.nih.gov/pubmed/30233248 http://dx.doi.org/10.2147/CMAR.S171223 |
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