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携带JAK2 V617F突变的中枢神经系统淋巴瘤，发生于真性红细胞增多症20年病史之后。

Central Nervous System Lymphoma Harboring the JAK2 V617F Mutation That Developed after a 20-year History of Polycythemia Vera.

作者信息

Kajikawa Natsuki, Seki Yoshimoto, Fujio Takayuki, Okoshi Yasushi, Hori Mitsuo, Saito Hitoaki, Iijima Tatsuo, Kojima Hiroshi

机构信息

Department of General Medicine, Ibaraki Prefecture Central Hospital, Japan.

Department of Primary Care and Medical Education, Faculty of Medicine, University of Tsukuba, Japan.

出版信息

Intern Med. 2018 Nov 15;57(22):3293-3297. doi: 10.2169/internalmedicine.1093-18. Epub 2018 Jul 6.

DOI:10.2169/internalmedicine.1093-18
PMID:29984752
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6287982/
Abstract

A 78-year-old man who had a 20-year history of polycythemia vera (PV) with a JAK2 V617F mutation presented with gradually progressive disturbance of consciousness. Hyper-intense lesions in the peri-lateral ventricular area and left cerebellar hemisphere were observed by T2-weighted and fluid-attenuated inversion recovery magnetic resonance imaging. Cytologic and genetic analyses of the lymphoma cells obtained from his cerebrospinal fluid established the diagnosis of B-cell lymphoma. No lesions outside of the brain were recognized. Because of his poor general condition, he was not treated actively. A postmortem analysis revealed a JAK2 V617F mutation in the lymphoma cells, suggesting their origin was a PV clone.

摘要

一名78岁男性，有20年真性红细胞增多症（PV）病史，伴有JAK2 V617F突变，出现逐渐进展的意识障碍。通过T2加权和液体衰减反转恢复磁共振成像观察到侧脑室周围区域和左小脑半球有高强度病变。对从其脑脊液中获取的淋巴瘤细胞进行的细胞学和基因分析确诊为B细胞淋巴瘤。未发现脑外病变。由于他的全身状况较差，未进行积极治疗。尸检分析显示淋巴瘤细胞中有JAK2 V617F突变，提示其起源于PV克隆。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/97fc6cc08218/1349-7235-57-3293-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/0f1a6d5324c7/1349-7235-57-3293-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/b0f6c0d46b94/1349-7235-57-3293-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/97fc6cc08218/1349-7235-57-3293-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/0f1a6d5324c7/1349-7235-57-3293-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/b0f6c0d46b94/1349-7235-57-3293-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f57/6287982/97fc6cc08218/1349-7235-57-3293-g003.jpg

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