颗粒性外周T及NK淋巴瘤 诊断鉴别诊断 山东泰安 柏世玉.pptxVIP

颗粒性外周T及NK淋巴瘤诊断/鉴别诊断山东泰安 柏世玉手机/微淋巴细胞超微结构? The few lysosomes in blood lymphocytescontain several different acid hydrolases,Acid hydrolase activities are generallyhigher in T cells than in non-T .? An example of such secretory lysosomesare the specialized cytoplasmic granulesof T cells and NK cells that are responsiblefor the cytotoxic effector function of thesecells.----引自Williams Hematology 2015 细胞毒T细胞及NK细胞结构及作用模式 细胞毒T细胞及NK细胞光学及超微结构 ? NK细胞和细胞毒性T细胞的胞浆中存在嗜苯胺蓝颗粒，这些颗粒包含细胞毒素蛋白,如T细胞胞浆内抗原(TIA-1)、Granzyme B、穿孔素(perforin)。? 粘膜和皮肤γδT细胞淋巴瘤：通常TIA-1、Granzyme B和perforin均为阳性；? ALCL：TIA-1、 Granzyme B、perforin也常为阳性；? 鼻型NK/T细胞淋巴瘤及ANKL淋巴瘤细胞：＞90%病例TIA-1和Granzyme B为阳性，60%为perforin阳性；? MF进展后期淋巴瘤细胞也能表达TIA-1抗原? T-LGL及LNK-CLPD：LGL细胞表达(TIA-1)、Granzyme B、perforin。? HSTCL： TIA-1常为阳性，Granzyme B和perforin阴性； 肿瘤性/反应性？T/NK？ 案例1 T-LGLL ? D. Bone marrow aspirate smear from a patient with γδ T-LGL leukemia showssmall to intermediate size lymphocytes with ample cytoplasm containingazurophilic granules and hyperchromatic nuclei. E. Bone marrow biopsy from apatient with γδ T-LGL leukemia shows normal cellularity and a subtlelymphoid infiltrate. F. Immunohistochemistry for CD3 highlights lymphocytesin an interstitial pattern in a patient with γδ T-LGL leukemia 案例2? 病史：心悸，入院淋巴结彩超示双侧腹股沟淋巴结肿大 T-LGLL与NK-CLPD形态学临床表现? 淋巴细胞一般轻至中度增 ? 约半数有脾大，少部分轻度多，或没有增多，但颗粒性淋巴细胞比例增高。肝大、淋巴结大，或有自身免疫性疾病症状。 T-LGLL相对明显/多见? 形态一般为胞质丰富，含有细致或粗大的嗜天青颗 ? 患者多数中度贫血、明显中粒，核圆形或轻度扭曲，染色质中等致密。性粒细胞减少、少见血小板减少，少数表现为纯红再障? T-LGL与NK-CLPD细胞形 ? 与LGL分泌FAS及配体导致态学无法区分 组织细胞凋亡有关 临床表现不同的案例3 颗粒性淋巴细胞百分比＞90%，于外院诊断为NK-CLPD 胞质丰富侧易见大小不一的嗜天青颗粒 LGLL免疫表型◆ Currently, the most commonly◆ T-LGLL and CLPD-NK cellsexpress TIA-1, perforin,andgranzyme B cytolytic granulesaccepted blood T-LGL cutofffor a diagnosis of LGLL isgreater than 0.5 × 109/L, witha TCRαβ+/CD3+/CD8+/CD57+immunophenotype, lasting morethan 6 months.◆ In contrast to normal LGL ofT-cell origin, leukemic LGLexpress significantly lowerlevels of CD5 and show◆ For CLPD-NK (CD3–/CD8+/CD16+ abnormal killer