Cobo Fernando, Talavera Paloma, Busquier Heriberto, Concha Angel
Department of Pathology and Tumor Bank, Infectious Pathology Unit, Hospital Universitario Virgen de las Nieves, Avda Fuerzas Armadas, 2,18014 Granada, Spain.
Neuropathology. 2007 Aug;27(4):396-402. doi: 10.1111/j.1440-1789.2007.00784.x.
We report a case of extranodal NK/T-cell lymphoma, nasal type, with exclusive cerebral localization in a patient with AIDS. The patient presented with neurological alterations, fever and convulsions, so the initial presumptive diagnosis was an opportunistic brain infection. MRI showed a left parietal necrotic lesion and a stereotactic brain biopsy was performed for pathological, microbiological and molecular studies. Histological sections showed an angiocentric and angiodestructive growth pattern and the immunophenotype of this tumor was CD56+, CD45+, CD3+ (cytoplasmic), Granzyme B+ and Perforin+. All the microbiological studies such as bacterial, fungi, micobacteria, Toxoplasma gondii and Cryptococcus determination were negatives. A PCR study with primer specific for EBV viral genome of Bam-Hi-w system was positive. Also, a rearrangement study showed T-cell gene rearrangement with monoclonal appearance. A diagnosis of extranodal NK/T-cell lymphoma was made and the patient died a few days later. This case represents a very rare example of NK/T-cell lymphoma of the brain in a patient with AIDS. The diagnosis of this kind of lymphomas requires a multimodality approach correlating clinical, morphological, immunophenotypic and molecular data.
我们报告一例艾滋病患者发生的结外NK/T细胞淋巴瘤，鼻型，仅累及脑部。该患者出现神经功能改变、发热和惊厥，因此最初的初步诊断为机会性脑部感染。MRI显示左侧顶叶坏死性病变，并进行了立体定向脑活检以进行病理、微生物学和分子研究。组织学切片显示血管中心性和血管破坏性生长模式，该肿瘤的免疫表型为CD56+、CD45+、CD3+（胞浆）、颗粒酶B+和穿孔素+。所有微生物学研究，如细菌、真菌、分枝杆菌、弓形虫和隐球菌检测均为阴性。用Bam-Hi-w系统EBV病毒基因组特异性引物进行的PCR研究呈阳性。此外，重排研究显示T细胞基因重排呈单克隆表现。诊断为结外NK/T细胞淋巴瘤，患者几天后死亡。该病例是艾滋病患者脑部NK/T细胞淋巴瘤非常罕见的一例。这类淋巴瘤的诊断需要结合临床、形态学、免疫表型和分子数据的多模态方法。
