间变性大细胞淋巴瘤

间变性大细胞淋巴瘤（ＡＬＣＬ）是形态学和免疫学上一种独特类型的非霍奇金淋巴瘤（ＮＨＬ）,它的发生与ｔ（２;５）（ｐ２３;ｑ３５）梁色体易位形成ＡＬＫ－ＮＰＭ融全基因有关。本文综述了ＡＬＣＬ在形态学、免疫学、遗传学和临床方面的特点及其诊断、治疗、预后。     

间变性大细胞淋巴瘤1例

间变性大细胞淋巴瘤(ALCL)临床较少见,约占非霍奇金氏淋巴瘤的2%～7%犤1犦,可累及淋巴结、皮肤、胃肠道等处,我科偶见1例,现报告如下。患者,女,34岁,因腹胀、右下肢水肿3个月于2002年6月3日入院。查体:双侧锁骨上、腹股沟、腋下均可触及肿大的淋巴结,质中,无压痛,与皮肤无粘连,最大约3cm×4cm,右腹股沟肿大淋巴结活动差,双腋下淋巴结相互融合,右侧乳房皮肤轻度水肿,双下肺呼吸音稍低,腹部膨隆,移动性浊音阳性,右下肢重度凹陷性水肿。查血常规:WBC25.1×109/L,N0.799,L0.076,M0.125,胸部正侧位片:两侧胸腔积液(少量)、右肺部感染,不除外…     

间变性大细胞淋巴瘤

198 5年Stein等[1] 首次描述间变性大细胞淋巴瘤(anaplasticlargecelllymphoma ,ALCL) ,并确认为一种新的淋巴瘤类型。ALCL具有下列特征 :肿瘤细胞呈间变性 ,生长具黏结成团倾向 ,侵犯淋巴结窦 ,间变细胞CD30 (Ki 1)强阳性。ALCL也称之为Ki 1阳性大细胞淋巴瘤。虽然ALCL具有上述共同特征 ,但病理形态、基因表型和临床表现有广泛的异质性 (表 1)。B细胞来源ALCL在REAL和WHO分类中归于弥漫性大B细胞淋巴瘤[2 ,3 ] 。文中如未提到细胞来源 ,则特指T/null细胞ALCL。　　一、病理ALCL诊断性病理特点为淋巴结结构部分消失 ,肿瘤…     

Ki—1阳性的间变性大细胞性淋巴瘤

报告３例Ｋｉ１阳性的间变性大细胞性淋巴瘤（ＡＬＣＬ）,几乎均以结外肿块为首发表现（椎骨、乳腺及腹膜后组织）,术前均考虑为非血液系统肿瘤。１例椎骨病变者,术中冷冻及术后初步病理检查均误诊为转移性癌。由于ＡＬＣＬ组织学表现较为特殊,推测以往被误诊的病例不在少数。结合３例的诊治情况,对ＡＬＣＬ的临床特点、组织学诊断及鉴别诊断等问题进行讨论。     

间变性大细胞淋巴瘤中ALK与clusterin的表达及其临床病理意义

背景与目的：间变性淋巴瘤激酶（ALK）是系统性间变性大细胞淋巴瘤（ALCL）较为特异的标志,近来研究发现一种新的蛋白clusterin在ALCL中也有较高的表达率,其可能在ALCL的发展中起作用,并对诊断和治疗具有潜在价值.本研究探讨ALCL中ALK及clusterin蛋白表达特点、相互关系及临床病理意义.方法：应用免疫组织化学EnVision法检测90例淋巴瘤组织中ALK及clusterin蛋白表达,其中包括47例ALCL及对照组周围T细胞淋巴瘤非特殊型（PTCL-u）22例,经典型霍奇金淋巴瘤（CHL）21例.结果：ALK在ALCL、PTCL和CHL中的阳性率分别为60%（28/47）、0和0;clusterin在三者中的阳性率分别81%（38/47）、27%（6/22）和14%（3/21）,ALK和clusterin在ALCL的阳性率均显著高于PTCL和CHL的阳性率（P＜0.05）,clusterin在ALK阴性的ALCL中的阳性率为68%（13/19）,也显著高于PTCL和CHL的阳性率（P＜0.05）.ALK阳性的ALCL中位年龄20岁（3～70岁）,显著低于ALK阴性者（P＜0.05）,阴性中位年龄48岁（4～71岁）,ALK阳性与否与发生部位、性别无关（P＞0.05）.clusterin的表达与否与年龄、部位和性别均无关（P＞0.05）.结论：ALK在ALCL中的特异性表达对其诊断、鉴别诊断并可能对临床预后判断具有重要价值.clusterin作为一新的分子标志物,在ALCL中的相对特异性高表达对ALCL的诊断、特别是对ALK阴性的ALCL与PTCL和CHL的鉴别诊断将具有重要意义.     

间变性淋巴瘤激酶阳性弥漫大B细胞淋巴瘤的研究进展

摘 要：间变性淋巴瘤激酶阳性弥漫大B细胞淋巴瘤（anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma,ALK+DLBCL）是弥漫大B细胞淋巴瘤(DLBCL)的一种罕见的亚型,具有特征性形态学、免疫表型和细胞遗传学特点。其临床过程具侵袭性,预后不良,明确认识该疾病是诊断的关键。选择性ALK抑制剂可能是ALK+DLBCL患者潜在的治疗选择。     

结肠ALK阴性的间变性大细胞淋巴瘤1例报道并文献复习

目的：探讨肠道原发性间变性淋巴瘤激酶（ ALK）阴性的间变性大细胞淋巴瘤（ anaplastic large cell lymphoma,ALK-negative,ALK-ALCL）的临床病理学特点、诊断及鉴别诊断。方法：收集1例结肠原发性ALK-ALCL病理标本,对其进行临床病理分析、免疫组织化学检测及EB病毒编码的RNA（ EBER）原位杂交检测并复习相关文献。结果：患者,男,35岁,因“间断解血便5年,腹痛2个月,发热1个月”入院。临床长期以肠结核、炎症性肠病（克罗恩病）治疗,一度有效。近一个月来,因反复发作高热,临床考虑肠穿孔,剖腹探查后行结肠次全切术。大体观察：结肠内可见直径3.5-9.0cm的溃疡3个,最大溃疡处可见穿孔。光镜下肿瘤组织弥漫浸润肠壁全层及其周围脂肪组织,可见较多“标志性”肿瘤细胞,形态符合普通型ALCL。免疫组织化学示肿瘤细胞弥漫表达CD30,表达T细胞抗原CD3,表达细胞毒性抗原穿孔素,ALK阴性。EBER原位杂交检测为阴性。结论：肠道原发ALK-ALCL是罕见的结外T细胞淋巴瘤,恶性度高,但因临床症状不具备特征性易导致临床误诊。诊断依据病理学形态特点及免疫组织化学指标综合判断。     

间变性淋巴瘤激酶阳性的间变性大细胞淋巴瘤研究进展

Stein于1985年首先报道间变性大细胞淋巴瘤(anaplas-tic large cell lymphoma,ALCL)是非霍奇金淋巴瘤(NHL)的一种独立类型[1]。最新的WHO分类中ALCL被归类于外周T细胞淋巴瘤,占同期NHL的2%~7%[2]。研究发现,约有半数患者产生致癌性的异常间变性淋巴瘤激酶(anaplasticlymphoma k     

原发皮肤型间变大细胞淋巴瘤研究进展

原发皮肤型间变大细胞淋巴瘤(PC-ALCL)是一种发生于皮肤的T细胞淋巴瘤,由间变性、多形性或免疫母细胞样大淋巴细胞组成,多数肿瘤细胞(>75%)表达CD30.现就近年来流行病学、生物学特征、临床表现、诊断和鉴别诊断以及治疗、预后等方面的研究进展进行综述.     

间变性大细胞淋巴瘤病理学进展

阐述了间变性大细胞淋巴瘤病理学、临床表现、免疫表型和分子遗传学等研究的进展。     

具有明显浆细胞特征的骨原发ALK阳性的间变性大细胞淋巴瘤1例报道及文献复习

目的:探讨1例具有明显浆细胞特征的骨原发ALK阳性的间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的临床病理学特征及鉴别诊断要点.方法:收集1例原发于右股骨远端的具有明显浆细胞特征ALK阳性的间变性大细胞淋巴瘤临床及影像学资料、观察其病理学形态及免疫表型,并复习相关文献,探讨该肿瘤的病理诊断和鉴别诊断要点.结果:患者为16岁男性,因右膝关节疼痛6月,加重伴功能障碍3周入院.MRI显示:右股骨下段骨质及周围软组织可见团片状长T1长T2信号影,压脂呈高信号影,骨质呈明显破坏表现,增强扫描病灶呈明显不均匀强化.镜下观察可见:肿瘤由大小较一致的浆样细胞组成,细胞异型性明显,核分裂象多见,浸润周围骨组织.免疫组化示肿瘤细胞表达CD30、ALK-1、CD4、EMA及GramB(+).结论:骨原发性ALCL非常罕见,具有明显浆细胞特征的ALCL形态学特殊,明确诊断需结合组织病理学及免疫表型综合分析.     

间变性大细胞淋巴瘤的临床病理与免疫组化研究(附3例报告及文献复习)

目的　研究间变性大细胞淋巴瘤 (ALCL)的临床病理和免疫组化特征。方法　应用免疫组化染色 (SP法 )对 3例ALCL进行免疫表型标记 ,采用了一种新的间变性大细胞淋巴瘤的特异性抗体ALKp80 (间变性淋巴瘤激酶 )。 结果　 3例ALCL的ALKp80均呈强阳性 ,2例CD30 阳性 ,2例LCA阳性 ,2例EMA阳性 ,1例CD685 0 %以上细胞阳性。 3例T、B细胞标记均不表达 ,CD15均呈阴性。结论　ALKp80不仅是ALCL的一个特异性标记物 ,而且对判断ALCL的预后有重要的意义。     

ALK阳性间变性大细胞淋巴瘤临床病理研究进展

约半数的间变性大细胞淋巴瘤（ALCL）病人中存在间变性淋巴瘤激酶（ALK）基因异常,ALK蛋白的异常激活使ALK阳性ALCL具有其典型的临床病理特征,并为ALK阳性ALCL的治疗提供新的靶点,提示ALK阳性ALCL的淋巴瘤可归类为一独立病种。     

16-Year Experience at M. D. Anderson Cancer Center with Primary Ki-1 (CD30) Antigen Expression and Anaplastic Morphology in Adult Patients with Diffuse Large Cell Lymphoma

One hundred and seven adult cases of untreated diffuse large cell lymphoma (DLCL) were retrospectively analyzed for primary CD30 antigen expression, anaplastic morphology, and long-term prognosis. Tissue samples from 36 patients stained strongly for CD30, and of these, 22 showed anaplastic morphology. Patients with CD30-positive DLCL presented more frequently with skin involvement, constitutional symptoms, more advanced Ann Arbor stage disease, and at a younger age when compared to patients with CD30-negative DLCL. Both groups had a similar complete response rate (78%). Patients with CD30-positive DLCL had a favorable survival rate when compared to patients with CD30-negative DLCL, although the difference was not statistically significant (73% vs. 55%, p = 0.28). Among the CD30-positive DLCL patients, those with anaplastic morphology presented more frequently with extranodal disease and T-cell phenotype when compared to patients with non-anaplastic morphology, but both groups had similar rates of complete response and long-term survival. All 107 patients were treated initially with a doxorubicin-containing chemotherapy regimen. These findings suggest a favorable outlook for adult patients with DLCL that has primary CD30 antigen expression regardless of the presence or absence of anaplastic morphology. Which support our previous observations.     

Breast implant-associated anaplastic large cell lymphoma: A comprehensive review

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a recently recognized non-Hodgkin lymphoma of T-cell origin. Despite the low incidence of this new disease, the increasing use of breast implants for cosmetic or post-mastectomy reconstruction purposes places BIA-ALC as an emerging and compelling medical challenge. The real BIA-ALCL pathogenesis has not been fully uncovered so far, while different putative causal factors have been proposed. Breast implants with textured surfaces seem to be associated with nearly all cases of BIA-ALCL, while the real the risk of disease development has not been well estimated so far. Late onset, persistent seroma around breast implant represents the classical clinical presentation. Most of the BIA-ALCL patients presents with localized disease, which confers an excellent prognosis. Unlike other non-Hodgkin lymphomas, surgical excision of the mass has a key role in the treatment. For patients with advanced and disseminated diseases, the treatment did not differ from other types of T-cell lymphoma. For these reasons, BIA-ALCL represents an emerging disease which requires multidisciplinary team approach to well define diagnostic workup and treatment for each patient. This review article aims to summarize available data on BIA-ALCL. First, we will outline available data on BIA-ALCL epidemiology, pathogenesis, diagnostic work-up, and treatment. Second, we will point out the potential psychological implications as well as the risk of perception distortion for women with breast implants, especially for those with previous breast cancer. Lastly, we will summarize the current national recommendations regarding textured breast implants and discuss the diagnostic-therapeutic algorithm for BIA-ALCL management.     

Research progresses in the pathogenesis of anaplastic large cell lymphoma

Anaplastic large cell lymphoma(ALCL) is a distinct subset of T-cell non-Hodgkin's lymphoma.As a consequence of its low incidence,general pathogenic consideration of ALCL is lacking.In this review,we summarize the pathogenesis,epidemiology,clinical manifestations,and treatment of ALCL,so as to better understand key stages of the development of this disease and provide valuable information for future treatment.     

ALK+弥漫大B细胞淋巴瘤的研究进展

ALK+弥漫大B细胞淋巴瘤(anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma, ALK+DLBCL)是弥漫大B细胞淋巴瘤的一种罕见的亚型, 具有特殊的免疫母细胞或浆母细胞样的形态学特点, 免疫表型独具特征, 细胞遗传学异常, 在儿童和成人都可发生。此病虽然罕见, 但是其临床过程具有侵袭性且预后不良, 因此明确认识该疾病的特点是诊断的关键。ALK+DLBCL对传统化疗方案反应性差, 最近推出的小分子ALK抑制剂可能对这种疾病的患者提供了一个潜在的新的治疗选择。      

Hodgkin-like anaplastic large cell lymphoma (previously designated in the REAL classification) has same immunophenotypic features to classical Hodgkin lymphoma

In the WHO classification, the majority of Hodgkin-like ALCL cases as defined by the REAL classification are considered to be CHL. However, establishing a histological diagnosis for the gray zone between CHL and ALCL is often confusing. In this study, we re-evaluated such cases by performing immunohistochemistry with antibodies against PAX-5/BSAP, Oct.2, and BOB.1/OBF.1. Expression of PAX-5/BSAP was observed in 88% (76/87) of CHL specimens and none (0/11) of ALK-positive ALCL specimens. Among specimens of Hodgkin-like ALCL and ALK-negative ALCL, expression of PAX-5/BSAP was observed in 77% (20/26) and 18% (3/17), respectively. Most of the PAX-5/BSAP-positive specimens were negative for Oct.2 and/or BOB.1/OBF.1 except for four CHL specimens. Our results may support the WHO classification in which most cases of Hodgkin-like ALCL are classified as CHL. However, the patients with Hodgkin-like ALCL with CHL-immunophenotype (PAX-5/BSAP-positive and negative for Oct.2 and/or BOB.1) did not have a favorable outcome, with a 5-year OS rate of 58%.     

富于粒细胞的间变性大细胞淋巴瘤(附六例报道并文献复习)

目的　探讨间变性大细胞淋巴瘤 (ALCL)的一种新类型—富于粒细胞的ALCL的临床病理特点。方法　收集 6例本院及会诊病例 ,免疫组化ABC法证实为ALCL ,使用抗体包括CD45,CD3 ,CD45RO,CD2 0 ,CD79,CD3 0 ,ALK1,EMA ,CD15,S -10 0 ,CD68,CD1a,均为DAKO产品。结果　 6例均原发于淋巴结 ,其中 1例为B细胞性 ,5例为T细胞性 ,强表达CD3 0 ,不表达CD15,组织学特点为在肿瘤细胞间有大量的嗜中性粒细胞和 (或 )嗜酸性粒细胞浸润 ,局部形成小脓肿。结论　富于粒细胞的ALCL具有独特的组织学特点 ,可能是ALCL一种新亚型 ,但临床应与Langerhans细胞组织细胞增生症 (LCH) ,霍奇金淋巴瘤 (HL) ,T细胞淋巴瘤及急性化脓性炎症鉴别。