nk细胞淋巴瘤讲义文档资料.ppt
,包括：结外NK/T细胞淋巴瘤，鼻型侵袭性NK细胞白血病慢性NK细胞淋巴增生性疾病,NK细胞的免疫表型特点,No single marker unequivocally identifies all NK cells,CD2 70-90%s-CD3 0%c-CD3 95%CD4 0%CD5 0%CD7 80-90%CD8 30-40%,CD11b*80-90%CD16*80-90%CD56*95%CD57*50-60%CD25 95%,*Commonly used NK markers,but expressed in both NK cellsand NK-like T cells,Discrepancy of CD3 expressionin NK cells,CD3 is a TCR-associated complexFormed by 6 chains:,2,2NK cells have cytoplasmic or,but cannot assemble intact CD3 complexConventional CD3 antibody detects chain“cytoplasmic CD3”,Surface CD3(as detected by Leu4/OKT3)by flow cytometry or on frozen tissue is NEGATIVE,TCR,Leu4,OKT3,结外NK/T细胞淋巴瘤，鼻型,a鼻腔、鼻咽部、鼻窦及腭部最常见，皮肤、软组织、胃肠道和睾丸亦常见。b鼻腔者表现为鼻塞、鼻衄，粘膜坏死及溃疡。皮肤病变表现为结节并多伴有溃疡。发生在肠道者常发生穿孔。c肿瘤常有噬血管性，多伴有血管破坏和坏死。d常坏死明显，活检取材要取及非坏死瘤组织，常需多次取材才能确诊。,大多数病例为EBV（+）、CD56（+）的NK细胞表型，而少数病例具有EBV（+）、CD56（-）的细胞毒性T细胞表型。,Small,Medium-sized,Large,Broad cytologic spectrum,Morphologic features,Surface squamous epithelium can show florid pseudoepitheliomatous hyperplasiaEntrapped nasal mucosal glands often show clear cell changeSkin:infiltrate often perivascular and periadnexal,but sometimes diffuse dermal;subcutis can be involvedIntestine:Ulceration and perforation common,Skin,Intestine,Testis,Muscle,结外NK/T细胞淋巴瘤的免疫表型CD2+表面 CD3-;胞浆 CD3+(polyclonal CD3;PS1)CD56+,CD57和CD16常-CD4,CD5,CD7,CD8:常-,CD43,CD45RO:常+细胞毒颗粒+Ki67:高CD25和 CD30偶尔+TCR-;,CD3,CD5,CD56,Granzyme B,Extranodal NK/T cell lymphoma:Genotype,TCR genes:germline in most cases studied,EBVPresent in clonal episomal formNasal:95%positive,irrespective ofethnic originExtra-nasal:90%positive in Asians,but lower%in Caucasians,Extranodal NK/T cell lymphoma:Main problems in diagnosis,Biopsies may show extensive coagulative necrosis,rendering it impossible to make a definitive diagnosis.Repeat biopsy is required.Some examples comprise small cells with minimal cytologic atypia:distinction from reactive lymphoid infiltrate very difficult,BEWARE:Mucosal small lymphoidcells often appear slightlylarger than small lymphocytes,and can show irregular nuclei,Histologic features favoring a neoplastic process,Abnormal architecture:Dense expansile infiltrate with wide separation and loss of mucosal glandsTissue ulceration and necrosisEvidence of invasion:Angiocentric growthCytologic atypia:Clear cytoplasmMedium-sized;nuclear irregularities;granular chromatinReadily found mitotic figures in a small cell lesion,Immunohistochemical analysis:First-tier panel,CD20,CD3,CD56,(Ki67),Nodular aggregates of B cells separated by T cells;few CD56+cells,Reactive lymphoidinfiltrate,Dense sheets of B cells,B celllymphoma,Dense sheets of CD3+CD56+cells,NK/T celllymphoma,Dense sheets of CD3+CD56-cells,Reactive orCD56-NK/Tlymphoma orT cell lymphoma,How to confirm a suspicion ofNK/T cell lymphoma?,Immunostain for CD56:large clusters or sheets of positive cells strongly support diagnosis of lymphoma Pitfall:herpes infectionIn-situ hybridization for EBER:large groups of positive cells support diagnosis of lymphomaImmunostain for Ki67:High index(80%)for a small cell-predominant lesion suggests lymphoma,Diagnosis more difficult for CD56-subset:Besides EBV+,need to demonstrate cytotoxic markers,谢谢,
