外周T细胞淋巴瘤非特指型的诊断学特征

Diagnostic features of peripheral T cell lymphoma, not otherwise specified

目的讨论外周T细胞淋巴瘤非特指型的临床及病理学特征、诊断及治疗方法。方法对2017年9月4日泰安市中心医院收治的1例50岁男性外周T细胞淋巴瘤非特指型患者进行病历分析并复习相关文献。结果患者50d前右侧耳后触及花生米大小的肿大淋巴结,3d前明显增大至鹌鹑蛋样大小,伴压痛。甲状腺激素及肿瘤标志物等检验指标均正常。彩色多普勒超声结果示双侧颌下区、双侧颈部、双侧锁骨上、双侧腋窝淋巴结肿大;双侧腹股沟区淋巴结稍肿大;腹腔淋巴结肿大;脾大。CT结果示右肺下叶斑片状阴影,双肺小结节,纵隔淋巴结肿大。骨髓形态示异常幼稚细胞(考虑为淋巴瘤细胞)占14.5%;骨髓流式结果可见约占35.09%的CD4阴性CD8阳性异常的T淋巴细胞;考虑为T细胞淋巴瘤白血病来源。病理显示倾向于外周T细胞淋巴瘤非特指型;免疫组化:CD3(+)、CD5(+)、CD2(+)、CD7(+)、CD45RO散在(+)、CD20(+)、CD23(+)CD21(+)、B细胞淋巴瘤基因2(BCL-2)点状(+)、CD56散在弱(+)、B细胞特异性激活蛋白(PAX)-5(-)、细胞周期性蛋白(CyclinD1)(-)、粒酶B(+/-)、穿孔素(-)、T细胞内抗原(TIA)(-)、Ki67约15%。结论外周T细胞淋巴瘤非特指型是淋巴瘤的少见类型之一,病情进展迅速,侵袭性强。目前多种治疗手段效果不理想,预后较差,期待新型药物的不断开发应用。

Objective To investigate the clinical and pathological features of peripheral T cell lymphoma, not otherwise specified (PTCL-NOS), and method of diagnosis and treatment. Methods The medical record of a 50-year-old man with PTCL-NOS admitted to Taian Central Hospital on September 4th, 2017 was analyzed and the related literature was reviewed. Results A peanut-sized lymph node behind the right ear was touched 50 days ago, and increased significantly to quail egg-like size with tenderness 3 days ago. Thyroid hormones and tumor markers were all in normal level. The results of color Doppler ultrasonography showed that bilateral submandibular, bilateral neck, bilateral supraclavicular and bilateral axillary lymph nodes were enlarged. Ultrasound examination also found bilateral inguinal lymph nodes slightly enlarged, abdominal lymph nodes enlarged and splenomegaly. CT showed patchy shadow on the lower lobe of the right lung, small nodules in both lungs, and enlarged mediastinal lymph nodes. Bone marrow morphology showed abnormal juvenile cells (considered as lymphoma cells) accounted for 14.5%;approximately 35.09% of CD4-CD8+ abnormal lymphocytes were found with flow cytometry;they were considered as T cell lymphoma leukemia origin. PTCL-NOS was determined with the pathological examination. Immunohistochemical results of lymph nodes biopsy showed that cluster of differentiation 3(CD3), CD5, CD2, CD7, CD23 (follicular dendritic cell, FDC), CD21 (FDC), CD20 (B Cell) were all positive;B-cell lymphoma 2(BCL-2), CD45RO, CD56 and Granzyme B were weakly positive;B cell specific activation protein5 (PAX-5), CyclinD1, perforin and T cell intracellular antigen were negative;and Ki 67 accounted over 15%. Conclusions PTCL-NOS is a rare type of lymphoma. It develops rapidly and has high invasion. At present, lots of treatment methods are not ideal and the prognoses are poor. The development and application of new drugs is imperative.