间变性大细胞淋巴瘤临床病理分析

目的：研究间变性大细胞淋巴瘤（ALCL）的临床病理特点。方法；在光镜下对ALCL分型，用免疫组化ABC法研究ALCL的免疫表型特点，使用的抗体有CD45、CD3、CD45RO、CD20、CD79、CD30、CD15、EMA、ALK1、CD68、S－100蛋白、CK、HMB45。结果：28例ALCL均强烈表达CD30，除5例为B细胞性外，18例为T细胞性，5例为裸细胞性。其中多形性7例，单形必7例（包括原发皮肤ALCL2例），淋巴组织细胞性4例，富于粒细胞性5例。结论：ALCL具有较广的细胞学范围，免疫组化在诊断与鉴别诊断中有重要作用。     

淋巴结外间变性大细胞淋巴瘤的病理学特性

一、材料与方法1.标本来源和临床资料 :5份Ｋｉ 1(ＣＤ30 )阳性间变性大细胞淋巴瘤 (Ｋｉ 1ＡＬＣＬ)标本 (乳腺 2例 ,胃 1例 ,皮肤 1例 ,骨髓 1例 )来自本院和解放军第一六一医院 1985～ 1998年间手术或活检病例 ,男 3份、女 2份 ,年龄最大者 5 8岁 ,最小者 19岁。首发症状为不明原因的发热。对其中 3例患者进行了随访 ,2例因复发或转移死亡 ,另 1例因胃腺癌死亡。 3例平均存活时间 3 .0 8年。2 .方法 :(1)免疫组织化学方法 :5份标本经 4%甲醛液固定 ,石蜡包埋切片 ,ＡＢＣ法染色 ,所用第一抗体 :Ｋｉ 1、ＣＤ15、ＣＤ2 0、ＣＤ45ＲＯ、白…     

胃肠道原发性间变性大细胞淋巴瘤临床病理分析

目的探讨胃肠道原发性间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的病理形态、免疫表型特征及临床预后。方法对5例胃肠道原发ALCL进行形态观察,免疫组化标记及随访,并结合相关文献进行讨论。结果本组ALCL男女之比为1.5∶1,平均年龄40.6岁。发生部位包括胃2例,胰、十二指肠1例,回盲部1例,结肠1例。所有病例均经外科手术切除,2例术后进行化疗。5例中有4例随访3至12个月,其中1例死亡,3例健在。ALCL形态表现多样,细胞呈多形性,大小不等,胞质丰富,细胞核大而不规则,呈扭曲、肾形、马蹄形及花环状,甚至见多核及巨核瘤细胞。免疫表型:5例ALCL均阳性表达CD30,部分表达EMA、CD3和CD45RO,而不表达CKpan、CD20、HMB45、CD68、CD15和CD117。结论 ALCL是一种少见的非霍奇金淋巴瘤,发生在胃肠道罕见,有必要与霍奇金淋巴瘤、弥漫性大B细胞淋巴瘤、低分化腺癌、胃肠道上皮样间质瘤、恶性纤维组织细胞瘤等进行鉴别。     

原发性纵隔大B细胞淋巴瘤临床病理分析并文献复习

目的：探讨原发性纵隔大B细胞淋巴瘤(primary mediastinal large B-cell lymphoma,PMBL)的临床病理学特点及诊断要点。方法：收集2010年9月~2014年12月病理确诊为PMBL的病例,对其对PMBL进行临床特点、病理形态学及免疫组织化学观察分析,并复习相关文献。结果：3例PMBL2例为男性,1例为女性,3例均侵犯邻近器官,2例伴颈部或锁骨上淋巴结受累,1例椎体受累(C7-T4)。镜下见不同程度的纤维化,瘤细胞呈巢状或弥漫浸润,瘤细胞胞质空亮丰富,细胞核圆形或卵圆形,其中1例可见坏死。免疫组织化学均表达CD20、CD79a、CD23、bcl2、CD23,其中2例表达CD30,均不表达CD3、CD5。随访3例均生存,化疗后1例获得CR,2例获得PR。结论：纵隔原发弥漫大B细胞淋巴瘤很少见,形态变化多端,容易引起误诊。提高对PMBL的认识,对避免误诊是至关重要的。     

原发淋巴结边缘区淋巴瘤临床病理分析

目的：研究淋巴结MZL形态特征、诊断要点和鉴别诊断，为临床治疗和预后提供依据，方法：采用常规制片、免疫组化ABC法标记，光镜观察。结果：10例淋巴结MZL男性6例，女性4例，以淋巴结缓慢增大为特征，而无肝脾肿大，外周血未见异常。病理形态分为：边缘区增生型2例，结节型4例，弥漫型2例和母细胞样型2型。细胞类型：CCL细胞型5例，MBC型6例，淋巴浆细胞型2例，母细胞样型2例，10例均经免疫组化证实。结论：淋巴结MZL与MALT型淋巴瘤形态、免疫表型和起源相似。由于淋巴结组织结构特点，MZL有特殊性。     

原发淋巴结套细胞淋巴瘤临床病理分析

目的：探讨原发淋巴结套细胞淋巴瘤（MCL）的临床病理与免疫组化特点。方法：收集6例淋巴结MCL,免疫组化ABC法确定肿瘤细胞特征,使用的抗体有CD45、CD20、CD79、CD45RO、CD30、CD68、TdT、CD43、CD5、cyclinD1、c-myc,IgD,IgM等。结果：光镜可将MCL分为4种亚型：套区型1例,结节型1例,弥漫型2例,母细胞化型2例。肿瘤细胞表达全B细胞标记,IgD CD43 ,cyclinD1(5/6),CD5(4/6) 。结论：MCL是一种具有特殊免疫表型的B细胞淋巴瘤,不同的组织学构型其预后可能不同,临床应与其它类型B细胞淋巴瘤鉴别,如淋巴结边缘区B细胞淋巴瘤（MZL）,滤泡性淋巴瘤（FL）及CLL／SLL等鉴别。     

皮肤原发性CD30阳性间变性大细胞淋巴瘤

目的 探讨皮肤原发性CD30阳性间变性大细胞淋巴瘤（ALCL）的临床及组织病理学特征,为病理诊断和鉴别诊断提供依据。方法 采用组织病理学及免疫组织化学SP法的白细胞共同抗原、CD20、CD30、CD45RO、CD68、上皮膜抗原、细胞角蛋白和HMB45染色对9例皮肤原发性CD30阳性ALCL进行观察。结果 患者年龄31-84岁（平均58.2岁）,男女之比2：1,均以皮肤丘疹或皮下包块就诊。组织形态;瘤细胞体积大,呈多形性、圆形或椭圆形,胞质丰富。核大,核仁明显,核分型象多,常见R-S样细胞和多核巨细胞,CD30阳性,其中6例同时表达CD45RO,非T非B型3例表达,随访：2例因肿瘤转移而死亡,2例肿瘤复发,5例无复发,健在。结论 皮肤原发性CD30阳性ALCL是具有独特形态特点及预后较好的肿瘤,根组织病理特征及CD30阳性,可与其他恶性肿瘤鉴别。     

淋巴结大B细胞淋巴瘤

1　临床资料患者女,24岁。因干咳2月,胸痛、胸闷、心慌气急1个月于1999年3月23日来我院门诊。1月底在无明显诱因下出现刺激性咳嗽,当地医院摄片发现纵隔肿块,未经治疗。2月底出现胸痛、胸闷、心慌气急,活动及平卧时加重。近来吞咽时有梗阻感,同时伴头部肿胀不适。发病以来无发热、盗汗现象。1995年曾因膝部良性骨巨细胞瘤而行局部切除术。体检:颈静脉怒张,右侧明显,胸壁见明显静脉网,呈上腔静脉压迫综合征。右锁骨上可扪及淋巴结4枚,蚕豆与黄豆大小,质稍硬,边界清,尚可活动。腹平软,肝脾不肿大,余未扪及肿大淋巴结。右锁骨上淋巴结细针穿刺…     

小儿间变性大细胞性淋巴瘤

目的 :探讨小儿间变性大细胞性淋巴瘤的临床、病理及预后。方法 :对 17例外检和尸检小儿间变性大细胞性淋巴瘤的临床资料、病理切片和随访结果进行分析。结果 :间变性大细胞性淋巴瘤占小儿非霍奇金淋巴瘤的 12 8% ;临床表现主要是外周淋巴结肿大及皮肤损害 ,长期反复发热常见 ;病理特征为淋巴结部分受累 ,成片异形大细胞侵犯淋巴窦及副皮质区 ,免疫组化CD30强阳性 ;预后相对较好。结论 :小儿间变性大细胞性淋巴瘤并不少见 ,需与恶性组织细胞增生症、T区或多形T淋巴瘤、霍奇金淋巴瘤、蕈样霉菌病和转移性癌等鉴别。CD30、CD15、LCA和EMA免疫酶标检查对诊断及鉴别诊断十分有用。     

肉瘤样型间变性大细胞淋巴瘤临床病理特征

目的探讨肉瘤样型间变性大细胞淋巴瘤(sALCL)临床病理特点、免疫表型及分子遗传学特征。方法对1例sALCL的临床、病理组织学、免疫表型及免疫球蛋白重链(IgH)和T细胞受体(TCR)基因克隆性重排情况进行观察并复习相关文献。结果眼观:送检淋巴结1枚,1.5cm×1.0cm×1.0cm,切面呈鱼肉状。镜检:淋巴结基本结构几乎完全被破坏,异型的梭形和上皮样细胞弥漫增生。免疫表型:瘤细胞呈CD30、ALK1、EMA、CD45RO、CD45、TIA1、granzymeB、perforin、CD68(部分)、SMA(梭形成分)阳性。基因重排:TCRβ1克隆性重排。结论sALCL属罕见恶性肿瘤,其形态不典型,易误诊为其他恶性肿瘤,免疫表型和遗传学异常有助于其诊断和鉴别诊断。     

系统性间变性大细胞淋巴瘤的免疫组织化学特征

目的:探讨系统性间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的免疫组织化学特征.方法:回顾性分析48例系统性ALCL的免疫组织化学和10例系统性ALCL原位杂交技术检测EBER(EBV-encoded small RNA)的结果.结果:48例系统性ALCL的肿瘤细胞均表达CD30,而间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)在41.7％(20/48)的病例阳性,其他指标阳性率为CD2为65.0％(26/40),CD3为36.2％(17/47),CD4为72.7％(16/22),CD5为42.9％(15/35),CD7为16.7％(5/30),上皮细胞膜抗原(epithelial membrane antigen,EMA)为65.6％(21/32),T细胞胞质内抗原(T-cell intracellular antigen-1,TIA-1)为79.2％(19/24),颗粒酶B(granzyme B-producing Breg,GrB)为70.0％(14/20).所有病例的B细胞标志(CD20,PAX5,CD79a)均阴性.10例系统性ALCL有2例出现部分肿瘤性大细胞EBER阳性.结论:CD30和ALK是诊断ALCL关键及较为特异的免疫指标;有时出现人类疱疹病毒第四型(Epstein-Barr virus,EBV)感染并不能排除ALCL的诊断.     

肺原发性淋巴瘤样肉芽肿型大B细胞淋巴瘤临床病理特点

目的探讨肺原发性淋巴瘤样肉芽肿（lymphomatoid granulomatosis,LG）型大B细胞淋巴瘤的临床病理特点、诊断与鉴别。方法分析1例肺LG的临床病理特点,行SP法免疫组化检测和原位杂交（EBER）,并复习文献。结果患者女性,54岁,临床上表现为双肺快速增多、增大的境界清楚的圆形结节,呈孤立性或弥漫性分布,并出现发热、体重减轻、全身无力等症状。胸腔镜肺活检组织示弥漫淋巴细胞及散在异形性大细胞浸润,并见血管受累和坏死,免疫表型：大细胞CD45＋,CD20＋＋,CD30＋,EBV＋,EBER＋,CD68＋;CD15、ALK、EMA、CD3、CD56等均-（散在小淋巴细胞CD3＋）。结论LG是一种罕见的原发于肺的淋巴瘤样肉芽肿型大B细胞性淋巴瘤,与EBV感染有关。诊断困难,临床上需要与Wegener肉芽肿、结核、结节病等肺结节性病变鉴别。病理学上也需与结核、非特异性肉芽肿病、霍奇金淋巴瘤及非霍奇金淋巴瘤等鉴别。经形态学、免疫表型与临床、影像学相结合可明确诊断。     

Differences in clinical behaviour and immunophenotype between primary cutaneous and primary nodal anaplastic large cell lymphoma of T-cell or null cell phenotype

The histological, immunophenotypic and clinical features of 19 primary cutaneous anaplastic large cell lymphomas (cutaneous ALCL) were compared with those of 18 primary nodal anaplastic large cell lymphomas (nodal ALCL) of T-cell or null cell type. Although cutaneous ALCL and nodal ALCL had identical morphological features, differences in surface marker expression and clinical behaviour were found. Immunophenotypical differences concerned the expression of epithelial membrane antigen (82% of the nodal ALCL were positive v. none of the cutaneous ALCL) and the cutaneous lymphocyte antigen (HECA-452), a possible skin-homing receptor on cutaneous T-lymphocytes (most tumour cells in 44% of cutaneous ALCL cases were positive, whereas nodal ALCL showed expression of HECA-452 on only few tumour cells (< 25%) in 18% of cases tested). Loss of T-cell markers was more pronounced for nodal ALCL. Patients with cutaneous ALCL were generally older (median 61 years) than patients with nodal ALCL (median 24 years) and, in contrast to the latter group, did not show bimodal age distribution. Survival after 4 years, using lymphoma-related death as an end-point, differed significantly between cutaneous ALCL and nodal ALCL; 92% for cutaneous ALCL and 65% for nodal ALCL ( P =0.04). The better survival of cutaneous ALCL patients could not be ascribed to differences in age, stage or initial mode of treatment. These data indicate that differences in ismmunophenotype and clinical behaviour exist between morphologically identical primary cutaneous and primary node-based ALCL. They indicate that the primary site is an important prognostic factor in predicting the clinical outcome of ALCL.     

原发于骨骼肌的间变性大细胞T细胞淋巴瘤

目的：探讨骨骼肌原发的间变性大细胞淋巴瘤的临床病理特征和免疫表型。方法：采用常规制片和免疫组化(S-P)法检测1例（14岁）骨骼肌原发的间变性大细胞淋巴瘤。结果：肿瘤细胞CD30、ALK-1、CD45RO和CD45阳性；而CD20、EMA、S-100蛋白、desmin和CD68阴性。结论：本例为间变性淋巴瘤激酶（ALK）阳性的间变性大细胞淋巴瘤。骨骼肌原发的间变性大细胞淋巴瘤非常少见，诊断旱应先排除其它肿瘤和其它部位淋巴瘤累及骨骼肌。     

Cytological features of breast implant‐associated anaplastic large cell lymphoma in pleural effusion

Breast implant‐associated anaplastic large cell lymphoma (BIA‐ALCL) is a very rare CD30‐positive ALK‐negative T‐cell non‐Hodgkin lymphoma included as a provisional entity in the 2017 WHO classification of lymphoid neoplasms. BIA‐ALCL arises as proliferating cells over the surface of the implant. It is generally an indolent disease if confined within the fibrous capsule. In contrast, mass and/or infiltration beyond the capsule is much more aggressive. This report describes a case of infiltrative BIA‐ALCL with massive pleural effusion containing hallmark BIA‐ALCL cells showing the characteristic morphologic appearance of high‐grade anaplastic lymphoma, CD30‐positive but ALK‐negative with variable staining for T‐cell antigens. Detailed cytological features of BIA‐ALCL in pleural fluid are described along with the results of a literature search performed for BIA‐ALCL cases with pleural effusion. This report expands the spectrum of BIA‐ALCL pathology to include chest wall involvement and pleural effusion.     

A small cell variant of ALK-positive, CD8-positive anaplastic large cell lymphoma with primary subcutaneous presentation mimicking subcutaneous panniculitis-like T-cell lymphoma

ALK-positive anaplastic large cell lymphoma (ALK+ ALCL) is an uncommon non-Hodgkin's lymphoma of T-cell origin, the majority of which express CD4 and show frequent pan-T-cell antigen loss. While most cases of ALK+ ALCL have the common pattern characterized by anaplastic morphology with hallmark cells, a less common but well-recognized variant with a small cell pattern may pose a diagnostic challenge. We report a case of ALK+ ALCL with small cell morphology and CD8 subset restriction in a 53-year-old male patient who presented primarily with multiple recurrent subcutaneous nodules with histopathologic features simulating a subcutaneous panniculitis-like T-cell lymphoma (SPTCL). The case was initially diagnosed as SPTCL but was reconsidered as ALK+ ALCL when the incidental finding of CD30 positivity on a subsequent biopsy prompted an ALK immunostain, which turned out to be positive in the neoplastic T-cells. The diagnosis of ALK+ ALCL, small cell variant, was then confirmed by detection of an ALK gene rearrangement by FISH analysis. This report highlights a case of ALK+ ALCL with a deceiving clinical and histopathologic presentation, and emphasizes the value of immunohistochemical panel studies and genetic tests in such cases to avoid diagnostic errors.     

Fine-needle aspiration biopsy of anaplastic large cell lymphoma, small cell variant with prominent plasmacytoid features: case report

Anaplastic large cell lymphoma (ALCL), according to the new WHO classification, is a diagnosis limited to T/NK cell lymphomas. We present a case that demonstrates a new morphologic variant of ALCL with significant possible pitfalls for the cytopathologist. A fine-needle aspiration biopsy of a cervical lymph node showed a cellular aspiration comprised of medium-sized plasmacytoid cells in a discohesive and focally loosely cohesive pattern. The cytologic diagnosis confirmed the presence of malignancy and noted the prominent plasmacytoid features. An accompanying comment favored melanoma and included a broad differential. No cell block was available for immunohistochemical stains. Immunophenotyping of the subsequent excisional node biopsy showed an anaplastic lymphoma kinase (ALK)-positive ALCL. This case illustrates a new variant of ALCL. Although ALCL variants, such as small cell and lymphohistiocytic, are well recognized, the plasmacytoid features are an additional potential source for misdiagnosis. This case report shows that a cytopathologist should include ALK-positive ALCL in the differential diagnosis of plasmacytoid proliferations cell because of the clinical importance of the ALK-positive ALCL.     

Extranodal diffuse large B cell lymphoma of cutaneous follicle centre lymphoma type: a study of 24 patients with non-cutaneous primary limited stage extranodal diffuse large B cell lymphoma in support of a new concept

Aigner F, Korol D, Schmitt A M & Kurrer M O
(2012) Histopathology  60, 774–784 Extranodal diffuse large B cell lymphoma of cutaneous follicle centre lymphoma type: a study of 24 patients with non‐cutaneous primary limited stage extranodal diffuse large B cell lymphoma in support of a new concept Aims: Follicle centre cell lymphoma of small cell type showing either a follicular or diffuse growth pattern similar to cutaneous follicle centre lymphoma (cFCL) has been recognized in extranodal non‐cutaneous sites. Our aim was (i) to investigate whether diffuse large B cell lymphoma (DLBCL) of cFCL type could be identified in extranodal non‐cutaneous sites and (ii) whether clinical characteristics similar to primary cFCL could be recognized. Methods and results: Of 24 extranodal non‐cutaneous DLBCLs, nine (38%) had large centrocytoid morphology and 15 (62%) were either ‘centrocytoid and centroblastic’ or ‘centroblastic and immunoblastic’. Six centrocytoid cases were Irf‐4 negative, Bcl‐6 positive and at most weakly CD10‐ or Bcl‐2‐positive by immunohistochemistry, consistent with DLBCL of cFCL type. All patients with cFCL type were stage IE and were significantly younger than other patients. Recurrences occurred in two patients and were exclusively extranodal. Conclusion: Our results suggest that DLBCL of cFCL type can be identified in extranodal non‐cutaneous sites and shows clinical characteristics similar to genuine cFCL. We propose to expand the concept of cFCL to encompass large cell lymphomas in extranodal sites.