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。     

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。     

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。     

女性阴阜肉瘤样型与富于巨细胞型混合性间变大细胞淋巴瘤一例

患者女,34岁。4个月前无意中发现阴阜肿物,约花生米大,自觉肿物生长迅速,近3d肿物触痛明显,于2005年12月26日入院就诊。体检：左侧阴阜距皮下0．5cm处可触及一直径约为3cm的肿物,界限清楚,触痛明显。B超示：左阴阜皮下囊肿,结节性质待查。血液学检查：外周血象未见异常,骨髓穿刺可见少数异形的淋巴细胞。遂行阴阜肿物切除术。     

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.     

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…     

间变性大细胞淋巴瘤形态学及免疫表型观察

目的：探讨间变性大细胞淋巴瘤（ALCL）的形态学和免疫表型特征。方法：对6例ALCL和2例弥温性大B细胞淋巴瘤（DLBCL）进行形态学和免疫组织化学染色（ABC法）观察。结果：6例ALCL中，普通型2例、淋巴组织细胞型2例、ALK－变型2例，均可见单型性或多形性的标志性大细胞。普通型和ALK－变型大细胞沿淋巴窦内生长，而淋巴组织细胞型大细胞则呈散在分布；2例DLBCL形态上颇似ALCL；6例ALCL均为T细胞，CD30＋，儿童患者共同表达ALK＋和EMA＋，年长者则ALK－和EMA－。2例DLBCL均为B细胞，ALK＋、CD30－和EMA－。结论：不论何型ALCL，均可见CD30＋的标志性大细胞，淋巴窦内生长多见于普通型和ALK－变型。ALCK均为T细胞，儿童常有ALK和EMA共同表达，年长者则ALK和EMA－。DLBCL的免疫表型不同于ALCL。     

间变性大细胞性淋巴瘤--浆细胞样小细胞变型

1　临床资料患者男 ,2 7岁。临床表现为左腹股沟包块伴皮肤破溃 ,查体发现 1 2ｃｍ× 0 8ｃｍ× 0 5ｃｍ的包块 ,在当地医院体检未发现明显异常 ,切除包块并送病检。标本经常规 10 %福尔马林液固定 ,石蜡包埋 ,切片 ,分别进行ＨＥ染色及免疫组织化学染色。病理诊断 :“高度疑为淋巴浆细胞性淋巴瘤” ,后来我院病理科会诊。2　病理检查2 .1　镜检　低倍镜下表现为淋巴结结构不清、完全破坏 ,瘤细胞侵犯淋巴结周围组织 ,瘤细胞呈弥漫性、浸润性、破坏性生长 ,局部区域出现大片坏死。高倍镜下见大部分瘤细胞排列疏松。细胞间缺乏粘附 ,局部区…     

原发性软组织大细胞间变型T细胞性淋巴瘤1例

患者女 ,31岁 ,因右大腿肿胀疼痛伴化脓感染 3个月 ,切开引流术后 1周持续高热入院。查体发现右大腿软组织内肿块约 11ｃｍ× 7ｃｍ ,局部皮肤红肿 ,引流口有黄色脓性液体。全身淋巴结未及肿大 ,腹部Ｂ超及胸片未查见明显包块阴影 ,大腿局部Ｘ线片及ＳＰＥＣＴ示肿块阴影未侵及骨组织 ,股骨正常。临床拟诊肉瘤伴感染 ,行局部第一次活检 ,化疗后行大体肿块切除检查 ,随访。病理检查　活检肿瘤组织 3ｃｍ× 2ｃｍ及化疗后切除肿瘤组织 11ｃｍ× 8ｃｍ× 6ｃｍ ,切面灰白、淡黄色鱼肉样 ,与周围组织界限不清。　　镜检 :活检瘤组织弥漫分布 ,大…     

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。     

Anaplastic large cell lymphoma involving the urinary bladder: A case report and review of the literature

T cell‐derived malignant lymphoma is rarely detected as a bladder neoplasm. A literature review for anaplastic large cell lymphoma (ALCL) involving urinary bladder reveals only seven previously reported cases. Here, we report a case of a 59‐year‐old HIV‐negative man with ALK‐positive ALCL. He presented an unusual clinical course with initial consideration of adult onset Still's Disease (AOSD) due to his negative results searching for malignancy and infectious diseases. He rapidly developed macrophage activation (hemophagocytic) syndrome and experienced an unusual rapid disease progression and died in 39 days after onset of symptoms. Compared to previously reported cases, the current case of ALK‐1‐positive ALCL is a rare case with an unusual presentation. From this case, we learned that ALCL is one malignancy that should be considered and screened in patients with suspected AOSD. Also, T‐cell lymphoma associated hemophagocytic syndrome should be considered in a patient with sustained corticosteroid‐resistant spike fever, high serum ferritin, and rapid exacerbation of the disease course. Diagn. Cytopathol. 2015;43:60–65. © 2014 Wiley Periodicals, Inc.     

Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature

Sarcomatoid variant of anaplastic large cell lymphoma (ALCL) is one of the rarest histologic variants of ALCL that consists of large, bizarre, often spindle-shaped, neoplastic cells resembling a soft tissue sarcoma. We report here such a case of ALCL with both pulmonary and multiple nodal involvement in a 47-year-old woman who initially presented with fever, cough, sputum, itching skin, and weight loss. The initial transbronchial lung biopsy showed discohesive pleomorphic malignant cells in a strong inflammatory milieu reminiscent of inflammatory malignant fibrous histiocytoma (MFH). Subsequent cervical lymph node biopsy revealed a spindle cell sarcoma predominantly composed of plump spindle and oval neoplastic cells in interweaving fascicles, with sparse inflammatory infiltrates, resembling pleomorphic-storiform type of MFH. However, these tumor cells in the lung and node lesions revealed essentially similar immunohistochemical features that were positive for CD30, EMA, TIA-1, granzyme B, and fascin, but negative for anaplastic lymphoma kinase (ALK), and T- or B-lineage-specific marker. The spindled cells stains diffuse strong positive for smooth muscle actin (SMA), along with vimentin. Further studies showed that the tumor produced large quantities of the proinflammatory cytokines interleukin-2 (IL-2), IL-6, and IL-8, which we believe may contribute to the pathogenesis of sarcomatoid transformation of this tumor, and was associated with the patient’s inflammatory symptoms. To the best of our knowledge, this is the first reported case of sarcomatoid variant of ALK-negative ALCL with null cell phenotype and in situ production of proinflammatory cytokines presenting as multiple nodes and pulmonary involvement.     

Primary cutaneous anaplastic large cell lymphoma occurring in an atopic dermatitis patient: a case report with review of the literature with emphasis on their association

Although the risk of malignant lymphoma in patients with atopic dermatitis (AD) remains controversial, an increased risk of malignant T-cell lymphoma in patients with AD has been reported. Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a relatively common distinct clinicopathological entity. However, occurrence of C-ALCL in patients with AD has been rarely reported. Herein, we describe the 5^th reported case of C-ALCL occurring in a patient with AD and review the clinicopathological features. A 30-year-old Japanese male with a long-standing history of AD presented with a gradually enlarged nodular lesion in the right abdominal wall, which had spontaneously regressed without therapy. Two years later, multiple nodular lesions appeared in his trunk, and swelling of multiple lymph nodes was also detected. Histopathological studies demonstrated diffuse proliferation of large-sized lymphocytes with large convoluted nuclei containing conspicuous nucleoli and relatively rich cytoplasm in the skin and lymph node. Immunohistochemically, these lymphocytes were positive for CD30, CD8, and MUM1, and negative for CD3, CD4, and ALK1. Accordingly, a diagnosis of primary C-ALCL was made. The patient died of disease after various courses of chemotherapy. Our clinicopathological review revealed that the prognosis of C-ALCL occurring in patients with AD is poor because two of 5 patients died of disease. Therefore, albeit extremely rare, AD patients with C-ALCL should be monitored closely, and additional clinicopathological studies are needed to clarify the pathogenesis of C-ALCL occurring in patients with AD.     

Anaplastic large cell lymphoma with histiocytic phenotypes

A 38 year old man died suddenly of widespread lymphoid neoplasm after a short clinical course. Histologically the tumor was composed of pleomorphic large cells that often demonstrated phagocytic activity. Moreover, histochemical and immunohistochemical tests revealed that the tumor cells had the phenotypes of both anaplastic large cell lymphoma and histiocytic cells. lmmunogenotyping failed to demonstrate any lymphoid nature. This case might be categorized as anaplastic large cell lymphoma with histiocytic phenotypes.     

ALK-negative anaplastic large cell lymphoma primarily involving the bronchus: a case report and literature review

Anaplastic large cell lymphoma (ALCL) is a mature T cell lymphoma with characteristic morphologic, immunophenotypic and cytogenetic features. Current WHO classification includes anaplastic lymphoma kinase (ALK)-positive and ALK-negative variants. ALCL rarely presents with obstructive symptoms of the main airway. In addition to reporting a HIV-associated bronchial ALK-negative ALCL in a 44 year-old female, our literature review identified eight cases of bronchial ALCL with several interesting clinicopathological features, including: 1) a female predominance (67%); 2) two thirds of patients younger than 18 years old; 3) uniformly presented with respiratory symptoms and progressed to respiratory failure; 4) the tumor involving the main airways; 5) often with localized disease at the initial presentation. This unusual presentation of ALCL may pose as a diagnostic pitfall and delay the treatment.     

淋巴瘤样肉芽肿型大B细胞淋巴瘤

目的 探讨淋巴瘤样肉芽肿型大B细胞淋巴瘤(原名淋巴瘤样肉芽肿病，lymphomatoid granulomatosis，LYG)的病理形态特征、病变性质及鉴别诊断要点。方法 对l例LYG的组织形态学、免疫组织化学、EBV原位分子杂交(EBER)结合临床特征进行了分析。结果 l例63岁男性患者，临床上表现为多系统多器官性病变，主要累及肺，表现为双肺内境界清楚的圆形结节，呈孤立性或弥漫性分布，并出现多发性皮下结节，发热、体重减轻、全身无力等症状。皮下结节活检示多个肉芽肿样结构，细胞形态多样，见组织样细胞、非典型淋巴样细胞、小淋巴细胞、浆细胞及散在多核巨细胞，可见一血管壁有淋巴样细胞浸润，未见明显中心粒细胞，可见核分裂象。肺部穿刺组织示弥漫淋巴样细胞浸润，并见灶性坏死，免疫表型示瘤细胞呈CD20 ，CD79α ，CD43 ,CD3-,GraB-，EBV散在 ，CK-，Syn-，原位杂交示EBER 。结论 本例LYG是一种罕见的淋巴瘤样肉芽肿型大B细胞性淋巴瘤，与EBV相关。临床与影像学上与Wegener肉芽肿相似。肺部出现结节状病灶时，临床上易与结核、肉芽肿病、肺癌及炎性假瘤等相混淆，病理上须与结核、非特异性肉芽肿病、结外的外周T细胞淋巴瘤等相鉴别，形态学、免疫表型结合临床特征可明确诊断。     

Anaplastic variant of diffuse large B‐cell lymphoma: Reappraisal as a nodal disease with sinusoidal involvement

Anaplastic variant (av) of diffuse large B‐cell lymphoma (DLBCL) is morphologically defined in the 2017 World Health Organization classification, but still an enigmatic disease in its clinicopathologic distinctiveness, posing the differential diagnostic problem from gray zone lymphoma (GZL) and classic Hodgkin lymphoma (cHL). Thirty‐one cases previously diagnosed as avDLBCL were reassessed. Of these, 27 (87%) and 4 (13%) were node‐based and extranodal diseases, respectively. They were further reclassified into nodal avDLBCL (n = 18), nodal CD30+ DLBCL with T‐cell/histiocyte‐rich large B‐cell lymphoma‐like features (CD30+ DLBCL‐THRLBCL) (n = 6), GZL with features intermediate between DLBCL and cHL (n = 3) and CD30+ extranodal DLBCL, NOS (n = 4). The nodal avDLBCL cases had a sheet‐like proliferation of large cells and/or Hodgkin/Reed‐Sternberg (HRS)‐like cells in 12 (67%) notably with a sinusoidal pattern in 16 (89%). They showed an expression of CD20 and/or CD79a in all and CD30 in 15 of 18. All of them were negative for PD‐L1 on tumor cells, although HRS‐like cells showed negativity or partial loss of other B‐cell markers to varying degrees. The present study highlighted the distinctiveness of the nodal avDLBCL with sinusoidal pattern, but without neoplastic PD‐L1 expression, which provide refined diagnostic criteria for a more precise pathologic and clinical characterization of this disease.