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
=========================================================================

● 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
=========================================================================

● 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)

间变性大细胞淋巴瘤

第一批免疫组化结果：CD45（+）、CD30（+）、ALK（-）、EMA（—）、CK（-）、Vimentin（+）。

确实难！考虑差不多，大细胞类淋巴瘤如间变T、弥漫大B、组织细胞类肿瘤如FDCS、组肉等等

ALK阴性的间变性大细胞淋巴瘤（ALCL）。谢谢楼主的好病例。

非常抱歉，自动免疫组化仪器今天出故障，免疫组化结果要推迟到明天，敬请谅解。

HL？

细胞团块状，不考虑淋巴瘤；

腹膜后肿物大小？此例没有免疫组化结果很难明确定位。但从形态上考虑淋巴造血细胞肿瘤

倾向于：（1）组织细胞肉瘤；（2）朗格汉斯细胞组织细胞肉瘤；（3）间变性大细胞淋巴瘤；（4）滤泡树突细胞肿瘤/肉瘤；（5）侵袭性B细胞瘤。

期待任兄的免疫组化结果，快点吧，等不急啦。

组织细胞肉瘤。

年轻人，背景细胞比较杂乱，肿瘤细胞核大，偏位，胞浆丰富，见多核，肾形核，考虑1.组织细胞肉瘤；2.间变大。

另外，3，4，5有种结节状分布样子，细胞核淡染，空泡状，和我们曾遇到的一例指状突树突细胞肉瘤有些相似，所以，做为一个鉴别诊断吧

耐心等IHC。

这种疑难的病例，只有学习的份了，呵呵。

朗格汉斯细胞组织细胞肉瘤首选

鉴别粒细胞肉瘤，但是没有明确的肿物。，CD117，CD43等结果？

期待免疫组化

图片所示即使是在同一个高倍的视野内，肿瘤细胞的大小和形态基本都相差太大，考虑1、外周T（多形T）细胞淋巴瘤。2、组织细胞肉瘤

 考虑组织细胞肉瘤

待 IHC

组织细胞肉瘤

第二批免疫组化今天下午做，预计晚上八点前可以有结果。