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性别 | 男 | 年龄 | 35岁 | 临床诊断 | |
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一般病史 | 发现肝功能异常5天,发热3天。 | ||||
标本名称 | 腹膜后淋巴结组织 | ||||
大体所见 | 腹腔镜夹取活检组织。 灰红不整形碎组织一堆,1X1X0.3CM。 |
本例反应性淋巴细胞组织细胞很多,所以CD68+吧!CD30如果是几乎全部区域(而不是局部区域)都是这样强度一致的膜点阳性,那么确实是ALCL ALK-型。ALCL ALK+型里面提到淋巴组织细胞变异型、小细胞变异型,而ALK-的ALCL提到其形态学与ALK+的一致,除了小细胞变异型外。所以,本例应该还是ALCL ALK-型,组织学形态本例也算淋巴组织细胞变异型吧,IHC的CD68强阳性的细胞偏小,大细胞(真正的肿瘤细胞)似乎弱阳性
Lymphoma - Non B cell neoplasms
T/NK cell disorders
Anaplastic large cell lymphoma, ALK negative
Reviewer: Dragos Luca, M.D.
Revised: 29 September 2011, last major update September 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.
Definition
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● ALK negative anaplastic large cell lymphoma (ALCL, ALK-) is included as a provisional entity, and is defined as a CD30+ T cell neoplasm that is not reproducibly distinguishable on morphologic grounds from ALK+ ALCL, but lacks ALK protein (WHO 2008)
Terminology
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● Previously included in the broader category of anaplastic large cell lymphoma
Epidemiology
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● Most frequently 40-65 years old
● Male predominance (M:F ratio 1.5:1)
● Most epidemiologic studies do not separate ALK+ from ALK- neoplasms
Sites
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● Frequent involvement of both lymph nodes and extranodal sites
● Usually nodal; extranodal involvement in skin, bone, soft tissue, GI tract
Etiology
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● Uncertain, some suggest it may be the final stage of histologic progression for a number of T cell lymphomas
Clinical features
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● Presentation with advanced stage III-IV disease in most patients
● Peripheral and/or abdominal lymphadenopathy, B symptoms, high International Prognostic Index
Treatment and prognosis
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● Significantly worse prognosis than ALCL, ALK+ with conventional therapy
● Better prognosis than peripheral T cell lymphoma NOS (ALCL ALK- vs. PTCL: 36% vs. 20%; overall survival: 49% vs. 32%,
Postulated normal counterpart
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● Activated mature cytotoxic T cell
Micro description
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● Generally effaced architecture by solid, cohesive sheets of neoplastic cells
● Intrasinusoidal infiltrate or within T cell areas if architecture is partially preserved (mimics metastatic carcinoma)
● Large pleomorphic cells, occasional prominent nucleoli, may have multinucleated, wreath-like and “hallmark” cells, higher N/C ratio than ALCL, ALK+
● May have sclerosis and eosinophilia
● Similar morphologic variants compared to ALCL, ALK+, except for small cell(即可以出现淋巴组织细胞变异型)
Cytology description
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● Deeply basophilic cytoplasm, prominent vacuoles, round or lobate nuclei, prominent nucleoli, clumped chromatin, multinucleation
Positive stains
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● Strong and diffuse uniform CD30 staining in all tumor cells (membrane and Golgi zone pattern, also cytoplasmic)
● Variable expression / loss of pan-T-cell antigens: CD2+ and CD3+ more often than CD5+
● Almost always CD43+
● Often CD4+, rarely CD8+
● TIA1, granzyme B, perforin, clusterin, fascin, rarely EMA
Negative stains
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● ALK, CD15, CD20, CD79a, cytokeratin, BCL2, PAX5/BSAP, PGM1, EBV (EBER & LMP1)
Genetics and Molecular
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● T-cell receptor (TCR) gene rearrangement in most cases, irrespective of T cell antigen expression
● No recurrent cytogenetic abnormality
Differential diagnosis
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● ALK positive ALCL: ALK+, younger age, less aggressive
● Primary cutaneous ALCL: much better prognosis, clinical correlation with staging necessary
● Classical Hodgkin lymphoma
● Peripheral T cell lymphoma, NOS: difficult differential, WHO recommends conservative approach (diagnose ALCL, ALK- only if very similar to ALCL, ALK+, except for ALK expression)
T细胞的标记做得少了些,最好把CD2,CD7,CD4,CD8,CD5都加上,你诊断就更放心了,CD45RO特异性不好,现在基本已经废弃
最困惑的可能是间变为什么CD68会阳,查了下文献,倒是有的。现在发现CD68能在很多肿瘤中表达
Division of Pathology, Istituto Nazionale di Ricovero e Cura a Carattere Scientifico, Aviano, Italy.
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