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一例伴有巨大淋巴结病的侵袭性系统性肥大细胞增多症患者对米哚妥林治疗有反应。

A case of aggressive systemic mastocytosis with bulky lymphadenopathy showing response to midostaurin.

作者信息

Sciumè Mariarita, Serpenti Fabio, Muratori Simona, Pravettoni Valerio, Ulivieri Fabio Massimo, Croci Giorgio Alberto, Migliorini Anna Chiara, Esposito Andrea, Goldaniga Maria Cecilia, Saporiti Giorgia Natascia, Galassi Giulia, Baldini Luca, Onida Francesco, Grifoni Federica Irene

机构信息

Hematology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy.

Università degli Studi di Milano Milan Italy.

出版信息

Clin Case Rep. 2020 Dec 29;9(2):978-982. doi: 10.1002/ccr3.3717. eCollection 2021 Feb.

DOI:10.1002/ccr3.3717
PMID:33598282
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7869399/
Abstract

Management of systemic mastocytosis is an emerging challenge which requires a multidisciplinary diagnostic approach and personalized treatment strategy. Midostaurin can rapidly reduce the disease burden, also in cladribine refractory cases.

摘要

系统性肥大细胞增多症的管理是一项新出现的挑战，需要多学科诊断方法和个性化治疗策略。米哚妥林可以迅速减轻疾病负担，对于难治性克拉屈滨的病例亦是如此。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/409f/7869399/c13eee124480/CCR3-9-978-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/409f/7869399/7a72195ba99d/CCR3-9-978-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/409f/7869399/c13eee124480/CCR3-9-978-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/409f/7869399/7a72195ba99d/CCR3-9-978-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/409f/7869399/c13eee124480/CCR3-9-978-g002.jpg

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Am J Hematol. 2019 Mar;94(3):363-377. doi: 10.1002/ajh.25371. Epub 2019 Jan 2.
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Consensus Opinion on Allogeneic Hematopoietic Cell Transplantation in Advanced Systemic Mastocytosis.晚期系统性肥大细胞增多症异基因造血细胞移植的共识意见
Biol Blood Marrow Transplant. 2016 Aug;22(8):1348-1356. doi: 10.1016/j.bbmt.2016.04.018. Epub 2016 Apr 27.
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The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia.2016 年版世界卫生组织髓系肿瘤和急性白血病分类。
Blood. 2016 May 19;127(20):2391-405. doi: 10.1182/blood-2016-03-643544. Epub 2016 Apr 11.
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Long-term efficacy and safety of cladribine (2-CdA) in adult patients with mastocytosis. cladribine（2-CdA）在成年肥大细胞增多症患者中的长期疗效和安全性。
Blood. 2015 Aug 20;126(8):1009-16; quiz 1050. doi: 10.1182/blood-2014-12-614743. Epub 2015 May 22.
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