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累及纵隔和肺的淋巴浆细胞淋巴瘤,继之发生淀粉样变性:1例经手术和基因证实的病例。

Lymphoplasmacytic lymphoma involving the mediastinum and the lung, followed by amyloidosis: A surgically and genetically proven case.

作者信息

Adachi Yuichi, Takimoto Takayuki, Takeda Maiko, Matsumoto Kinnosuke, Takeuchi Naoko, Kagawa Tomoko, Sakamoto Tetsuki, Kasai Takahiko, Sugimoto Chikatoshi, Inoue Yasushi, Tachibana Kazunobu, Arai Toru, Inoue Yoshikazu

机构信息

Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-Cho, Kita-Ku, Sakai City, Osaka, 591-8555, Japan.

Department of Pathology, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-Cho, Kita-Ku, Sakai City, Osaka, 591-8555, Japan.

出版信息

Respir Med Case Rep. 2020 Dec 1;31:101313. doi: 10.1016/j.rmcr.2020.101313. eCollection 2020.

DOI:10.1016/j.rmcr.2020.101313
PMID:33318921
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7723813/
Abstract

A 60-year-old man was admitted for ground glass opacity in the lower lung field and mediastinal lymphadenopathy. Blood tests revealed elevated serum IgM levels, and the urine test detected Bence-Jones protein. Surgical biopsy from the mediastinal lymph node and lung showed small lymphocytes and plasma cells between follicles, and AL kappa amyloid deposition. Genetic examination detected L265P mutation. Our diagnosis was lymphoplasmacytic lymphoma (LPL), involving the mediastinum and the lung, followed by amyloidosis. Mutation analysis, in addition to conventional histological evaluation, was useful for a precise diagnosis.

摘要

一名60岁男性因下肺野磨玻璃影及纵隔淋巴结肿大入院。血液检查显示血清IgM水平升高,尿液检查检测到本周氏蛋白。纵隔淋巴结和肺部的手术活检显示滤泡间有小淋巴细胞和浆细胞,以及ALκ淀粉样蛋白沉积。基因检测发现L265P突变。我们的诊断是淋巴浆细胞淋巴瘤(LPL),累及纵隔和肺部,继发淀粉样变性。除了传统的组织学评估外,突变分析有助于精确诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/4f862a1f62c8/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/fe53fe35b581/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/351a654b531c/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/7126d35c8131/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/fd9e9e97534c/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/4f862a1f62c8/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/fe53fe35b581/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/351a654b531c/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/7126d35c8131/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/fd9e9e97534c/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e182/7723813/4f862a1f62c8/gr5.jpg

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本文引用的文献

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MYD88 L265P mutation analysis helps define nodal lymphoplasmacytic lymphoma.MYD88 L265P 突变分析有助于明确结内淋巴浆细胞淋巴瘤。
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Mutations in TLR/MYD88 pathway identify a subset of young chronic lymphocytic leukemia patients with favorable outcome.TLR/MYD88 通路突变可鉴定出具有良好预后的年轻慢性淋巴细胞白血病患者亚群。
Blood. 2014 Jun 12;123(24):3790-6. doi: 10.1182/blood-2013-12-543306. Epub 2014 Apr 29.
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Activation of TAK1 by MYD88 L265P drives malignant B-cell Growth in non-Hodgkin lymphoma.TAK1 被 MYD88 L265P 激活驱动非霍奇金淋巴瘤中恶性 B 细胞的生长。
Blood Cancer J. 2014 Feb 14;4(2):e183. doi: 10.1038/bcj.2014.4.
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A mutation in MYD88 (L265P) supports the survival of lymphoplasmacytic cells by activation of Bruton tyrosine kinase in Waldenström macroglobulinemia.MYD88(L265P)突变通过激活沃尔登斯特伦巨球蛋白血症中的布鲁顿酪氨酸激酶支持淋巴浆细胞的存活。
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Incidence of lymphoplasmacytic lymphoma/Waldenström's macroglobulinaemia in Japan and Taiwan population-based cancer registries, 1996-2003.1996-2003 年日本和中国台湾地区人群癌症登记处淋巴浆细胞淋巴瘤/华氏巨球蛋白血症的发病情况。
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