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经开放性肺活检诊断的肿瘤性血管内皮瘤病

[Neoplastic angioendotheliosis diagnosed by open lung biopsy].

作者信息

Matsushita A, Ishikawa T, Nagai K, Kubo T, Tanaka H, Uchida H, Takahashi T

机构信息

Department of Immuno-Hematology, Kobe City General Hospital.

出版信息

Rinsho Ketsueki. 1995 Mar;36(3):224-6.

PMID:7783326
Abstract

Neoplastic angioendotheliosis (NAE) is a rare neoplastic disease, and its pre-mortem diagnosis is extremely difficult. A 49-year-old male developed vertigo, hearing and visual disturbance, transverse myelopathy below Th 5 and hypercalcemia. These symptoms were markedly improved by VEPA chemotherapy. Thirty-four months after onset, diffuse reticular shadows were noted on chest X-ray. The biopsy specimen of the lung revealed intravascular lymphoid cells differentially stained with L26 and LCA. Southern blot analysis of the DNA from the tissue showed rearranged bands for the immunoglobulin gene (JH). A diagnosis of NAE of B-cell nature was established.

摘要

肿瘤性血管内皮细胞增生症(NAE)是一种罕见的肿瘤性疾病,其生前诊断极为困难。一名49岁男性出现眩晕、听力和视觉障碍、T5以下横贯性脊髓病以及高钙血症。这些症状通过VEPA化疗得到明显改善。发病34个月后,胸部X线显示弥漫性网状阴影。肺活检标本显示血管内淋巴细胞经L26和LCA差异染色。对该组织的DNA进行Southern印迹分析显示免疫球蛋白基因(JH)有重排条带。确诊为B细胞性质的NAE。

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