胃肠道的单形上皮性T细胞淋巴瘤(MEITL)是一种侵袭性T细胞淋巴瘤,很少涉及大脑。我们详细介绍了一名罕见的老年妇女的临床和尸检神经病理学发现,该妇女接受了小肠MEITL手术和化疗。手术后,在治疗结束时,她逐渐表现出神经系统衰退。患者最终死于疾病,尸检发现MEITL颅内受累。进行脑部尸检,并在100X放大倍数的光学显微镜下用苏木精-伊红染色检查组织。CD3,CD4,CD5,CD7,CD8,CD56,CD20,β-淀粉样蛋白,c-Myc,TCR-β,TCR-delta,对福尔马林固定石蜡包埋(FFPE)的脑组织进行EBER-ish。神经病理学检查显示,幕上区域多灶性脆性坏死和出血区域。组织学上,单调的中小型非典型淋巴细胞浸润脑实质,在船只周围突出。非典型淋巴细胞的免疫表型对CD-3,CD-7和CD-56呈阳性,对CD-5,CD-4,CD-8,CD-20和c-Myc呈阴性。EBER-ish为负。组织学和免疫表型证实了MEITL脑受累。已知MEITL患者的神经系统衰退和认知变化可能是大脑受累的第一个线索,因此需要及时进行评估。
Monomorphic epitheliotropic T-cell lymphoma (MEITL) of gastrointestinal tract is an aggressive T-cell lymphoma that can rarely involve the brain. We present detailed descriptions of clinical and autopsy neuropathological findings of a rare
case of an elderly woman who had surgery and chemotherapy for MEITL of the small intestine. Following her surgery, she progressively exhibited neurologic decline towards the end of her treatment. The patient eventually succumbed to her illness and was found to have MEITL with intracranial involvement on autopsy. Brain autopsy was performed and examination of tissues with hematoxylin-eosin staining under optical microscopy with 100 X magnification. Immunostaining for CD3, CD4, CD5, CD7, CD8, CD56, CD20, beta-amyloid, c-Myc, TCR-beta, TCR-delta, and EBER-ish was conducted on the formalin-fixed paraffin-embedded (FFPE) brain tissues. A neuropathological exam revealed multifocal friable necrotic and hemorrhagic areas in the supratentorial region. Histologically, monotonous small to medium-sized atypical lymphocytes infiltrated the brain parenchyma, prominently around the vessels. The immunophenotype of the atypical lymphocytes was positive for CD-3, CD-7, and CD-56 and negative for CD-5, CD-4, CD-8, CD-20, and c-Myc. EBER-ish was negative. The histology and immunophenotype confirmed the MEITL brain involvement. Neurologic decline and cognitive changes in patients with known MEITL can be the first clue of brain involvement upon which prompt evaluation is warranted.