间变性淋巴瘤激酶阴性的间变性大细胞淋巴瘤1例

<正>间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)阴性的间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)极为少见,我院近期收治1例,现已完成1个周期CDOP方案(环磷酰胺CTX Cyclophosphamide 1.0,d 1、脂质体阿霉素PLD Pegylated Liposomal Doxorubicin 30 mg,d 1、长春地辛VDS Vindesine 3 mg,d 1、地塞米松片DEX Dexamethasone,15mg,d 1)及5个周期CHOP化疗方案(环磷酰胺CTX Cyclophosphamide 1.0,d 1,吡柔比星THP theprubicin,     

12例间变性淋巴瘤激酶阳性间变性大细胞淋巴瘤临床分析

目的:分析间变性淋巴瘤激酶(ALK)阳性间变性大细胞淋巴瘤(ALCL)的临床特征,探讨治疗效果、预后影响因素及临床治疗方案。方法:收集2014年12月—2021年12月在徐州医科大学附属医院初治的12例ALK⁺ALCL患者的临床资料,回顾性分析其临床特点、治疗效果及预后生存情况。结果:12例患者的中位发病年龄为28(14～45)岁,其中男11例。11例(91.67%)患者初诊时评估晚期(Ⅲ～Ⅳ期),7例(58.33%)患者合并B症状,5例(41.67%)患者LDH升高,4例(33.33%)患者以浅表淋巴结肿大为首发部位,7例(58.33%)患者伴有腹腔淋巴结肿大。6例(50.00%)患者伴有结外侵犯,3例(25%)患者≥2个结外部位侵犯。2例(16.67%)患者IPI评分为高中危/高危组(3～5分);7例(58.33%)患者aaIPI评分为高中危/高危组(3～5分)。Ki-67≥80%、结外侵犯≥2处、aaIPI 3～5分、一线化疗方案是影响初始化疗后达CR的不良因素。CRP升高、结外侵犯≥2处、IPI 3～5分、aaIPI 3～5分、Ki-67≥80%、一线化疗...     

间变性大细胞激酶阴性CD30阳性原发性皮肤间变性大细胞淋巴瘤7例临床分析

目的 探讨间变性大细胞激酶(ALK)阴性CD30阳性(ALK^-CD30⁺)原发性皮肤间变性大细胞淋巴瘤(PC-ALCL)患者的临床特征。方法 回顾性分析7例ALK^-CD30⁺PC-ALCL患者的临床资料、临床表现、治疗方案、预后情况。结果 7例ALK^-CD30⁺PC-ALCL患者中,4例接受系统化疗,2例接受局部放疗;6例存活,其中1例拒绝治疗;1例死亡。根据患者病灶范围,区域型2例、区域型伴局部淋巴结浸润2例、广泛型3例。患者治疗总反应率为83.3%。2例区域型病灶患者通过局部放疗带病生存;2例区域病灶伴局部淋巴结浸润患者接受系统化疗后未出现化疗相关的严重并发症,达到无病生存;3例广泛型患者对化疗不敏感,其中1例死亡,2例带病生存。3例顽固性溃疡合并软组织感染的患者反复抗感染治疗无效,接受系统化疗后溃疡逐渐愈合。结论 ALK^-CD30⁺PC-ALCL患者需要根据病灶特点采取针对性治疗方案,区域型病灶、反复溃疡合...     

间变性大细胞淋巴瘤

198 2年Ｓｔｅｉｎ发现Ｒｅｅｄ Ｓｔｅｒｎｂｅｎｇ细胞示ＣＤ30 (Ｋｉ 1 ,Ｂｅｒ Ｈ2特异性单抗 )阳性 ,后来在部分非霍奇金淋巴瘤 (ＮＨＬ)亦见到Ｋｉ 1阳性细胞 ,称之为Ｋｉ 1阳性大细胞淋巴瘤〔1〕。并显示多形性大细胞增殖 ,特有的副皮质生长类型 ,局部坏死和显著的窦状隙弥散的病理学特征 ,而称之为间变性大细胞淋巴瘤 (ａｎａｐｌａｓｔｉｃｌａｒｇｅｃｅｌｌｌｙｍｐｈｏｍａ ,ＡＬ ＣＬ) ,成为具有共同的临床病理学特征的实体。这些病例原先已被诊断为恶性组织细胞增生症或间变性癌〔2〕。1　分类1 985年以前的恶性淋巴瘤工作分…     

原发系统型间变性大细胞淋巴瘤48例临床特征及预后分析

目的:探讨原发系统性间变大细胞淋巴瘤(ALCL)患者的临床特征及预后影响因素.方法:回顾性分析2013年1月-2021年3月于北京协和医院诊治的48例ALCL患者的临床资料.结果:48例患者中男31例,女17例,中位发病年龄37(18～79)岁.ALK+ ALCL 14例(29.2％),ALK-ALCL 34例(70....     

29例原发系统性间变大细胞淋巴瘤的病理特点及临床分析

目的:探讨原发系统性间变大细胞淋巴瘤(ALCL)的病理特点、临床特征与预后的关系.方法:对29例原发系统性ALCL患者的病理特点、临床特征、完全缓解率(CR)、长期生存率和预后因素进行了分析.结果:所有病例的瘤细胞均强表达CD30,58.62%间变性淋巴瘤激酶(ALK)阳性.接受了治疗的28例患者的CR率为50.0%,2年和5年生存率分别为51.3%与27.1%.ALK阳性患者CR为68.8%,2、5年无病生存率分别为60.2%、42.5%,明显高于ALK阴性组(P<0.05).国际预后指数(IPI)0～2分患者组CR率为57.1%,2年、5年无病生存率分别为80.2%、65.1%,明显高于IP13～5分患者组(P<0.05).Cox比例风险回归分析表明ALK的表达、IPI评分对预后的影响有统计学意义(P<0.05).结论:ALK表达、IPI评分有助于判断原发系统性ALCL的预后,并为个体化治疗提供了依据.     

间变性淋巴瘤激酶阳性大B细胞淋巴瘤的研究进展

间变性淋巴瘤激酶(ALK)阳性的大B细胞淋巴瘤(ALK+LBCL)是一种罕见且具有高度侵袭性的非霍奇金淋巴瘤亚型,患者确诊时多处于晚期,对CHOP样方案联合放疗及造血干细胞移植(HSCT)反应较差、预后不良.ALK抑制剂克唑替尼(crizotinib)、ALK嵌合抗原受体T(CAR-T)细胞治疗、ALK抗体及B细胞淋巴...     

以呼吸系统症状为首发表现的间变性大细胞淋巴瘤一例及文献复习

目的 提高对以呼吸系统症状为首发表现的间变性大细胞淋巴瘤（ALCL）的临床、实验室检查、胸部影像学和病理改变的认识。方法 对1例经纤维支气管镜肺活检和骨髓活检证实的ALCL病例的临床表现、实验室检查、影像学和病理资料并结合相关文献进行回顾性分析。结果 以呼吸系统症状为首发表现的ALCL症状主要表现为咳嗽、胸痛、咳血痰、气促、发热;实验室检查可有三系血细胞减少、脾大、骨髓噬血细胞增多;胸部X线和高分辨率CT（HRCT）早期可表现双肺多个小点状结节影,后可出现肺不张、肺团块影和纵隔淋巴结肿大,晚期可出现大量胸腔积液。ALCL的病理表现为肿瘤细胞体积大,核大而不规则,细胞弥散或巢样沿淋巴窦或滤泡间浸润,几乎所有的瘤细胞均表达间变细胞CD30（Ki-1）抗原。结论 以呼吸系统症状为首发表现的ALCL非常罕见,其临床表现、影像学检查无特异性,而诊断需要依靠病理,CD30（ki-1）阳性是诊断ALCL的有力依据。     

23例间变性大细胞淋巴瘤临床特征及预后分析

目的探讨间变性大细胞淋巴瘤（ALCL）的临床特征和免疫组化特点,并分析其预后因素。方法收集23例ALCL患者的临床资料。从临床特征和预后因素等几方面进行回顾性分析。结果本文完全缓解率68.75%,3 a生存率为60.82%;单因素分析显示,Ann Arbor分期、LDH及国际预后指数（IPI）与疾病预后相关。IPI≥3是ALCL的独立的预后不良因素。结论Ann Arbor分期、LDH与国际预后指数对预测患者长期生存和指导治疗有重要意义。     

Anaplastic lymphoma kinase‐positive anaplastic large cell lymphoma: current and future perspectives in adult and paediatric disease

Anaplastic large cell lymphoma (ALCL) is a rare T‐cell lymphoma seen in both adults and children. ALCL is associated with a characteristic chromosomal translocation, t(2;5)(p23;35) which fuses the anaplastic lymphoma kinase (ALK) gene on chromosome 2 with the nucleophosmin (NPM) gene on chromosome 5, resulting in a NPM‐ALK fusion protein, ALK over‐expression and constitutive tyrosine kinase activity. This aggressive lymphoma is more prevalent in males and can present with extranodal involvement (lung, skin and marrow infiltration) and haemophagocytic lymphohistocytosis. The long‐term overall survival is approximately 70–90% in children and over 70% in adults. Staging systems and prognostic risk factors are different in both childhood and adult ALCL. Treatment in adults is typically anthracycline‐based, with autologous stem cell transplantation (ASCT) salvaging patients in relapsed disease. There is evidence for ALL‐like therapy or intensive, pulsed anthracycline‐based induction in children. ASCT, allogeneic SCT and vinblastine maintenance are all considered reasonable options in relapsed childhood disease. The anti‐CD30 immunoconjugate Brentuximab Vedotin and the specific ALK inhibitor Crizotinib are changing the treatment paradigm in ALCL (ALK‐positive or negative) and ALK‐positive ALCL respectively. Both agents have shown encouraging responses in relapsed ALCL. It remains to be seen how these novel agents are used, but it is very possible that they may improve overall responses and survival in both children and adults. This review highlights the presentation, histopathological features, prognostic factors, and evidence‐based treatment approaches in the first line and relapsed setting in ALK‐positive ALCL. The review concludes by discussing the novel approaches using Brentuximab and Crizotinib which are being tested in clinical trials.     

Anaplastic large cell lymphoma in Japanese children: retrospective analysis of 34 patients diagnosed at the National Research Institute for Child Health and Development

This report presents a retrospective study of 34 Japanese children diagnosed at a single laboratory as having anaplastic large cell lymphoma (ALCL). Most of the pathological features of the Japanese ALCL children observed, such as anaplastic lymphoma kinase protein expression, in addition to most of the clinical characteristics and the outcomes of the patients, are very similar to those reported by European groups. To our knowledge, this is the first report describing the details of the clinicopathological features of Japanese ALCL patients. A further International study, including Japanese patients, might contribute to the establishment of an optimal treatment for paediatric ALCL.     

Anaplastic large cell Ki-1 lymphoma involving bone marrow: marrow findings and association with reactive hemophagocytosis.

This report describes the bone marrow findings in four patients whose marrow was involved by anaplastic large-cell Ki-1 lymphoma, an uncommon event in this type of lymphoma. In the marrow aspirate smears, the involvement was subtle, and was in the form of isolated large cells with irregular nuclear configuration, coarse chromatin, prominent nucleoli, and basophilic cytoplasm which might be vacuolated. One case showed paradoxically massive involvement in the trephine biopsy taken from the same site as the marrow aspirate. Reactive histiocytic proliferation with hemophagocytosis was also present. Since marrow aspirate or biopsy may be the first pathologic specimen examined in patients having anaplastic large-cell Ki-1 lymphoma, it is important to be able to recognize the small population of neoplastic cells, which should lead to prompt treatment or further investigations as deemed necessary.     

Pretargeted antibody-guided radioimmunotherapy in a child affected by resistant anaplastic large cell lymphoma

Anaplastic large cell lymphoma (ALCL) is characterized by preferential paracortical and intrasinusoidal lymph node involvement by large anaplastic tumor cells expressing the CD30 antigen. Up to 80% of pediatric patients with ALCL can be cured with multi-agent chemotherapeutic regimens. Patients resistant to chemotherapy or suffering from early relapse have a poor prognosis and a poor chance of survival. In these cases, the highly aggressive clinical course of ALCL, associated with systemic symptoms and extranodal involvement, has been treated with different approaches in various cooperative trials, including conventional chemotherapy and human stem cell transplantation (HSCT). However, the optimal treatment has not yet been defined, in particular in cases of relapse. More recently, radioimmunotherapy has been studied with encouraging results in cancer patients, including non-Hodgkin's lymphoma. Here we describe the case of a pediatric ALCL, relapsing after HSCT, treated with pretargeted antibody-guided radioimmunotherapy, obtaining a complete remission, with excellent quality of life over the past 10 months.     

Anaplastic large cell lymphoma (CD30+/Ki-1+): results of a prospective clinico-pathological study of 69 cases

Summary. Sixty-nine anaplastic large cell lymphomas (ALCLs) were selected from an Italian comparative trial on MACOP-B and F-MACHOP. As no significant difference in effectiveness of the protocols emerged, they were considered homogenously treated. The ALCLs were divided into two groups according to previously defined criteria: 41 were common type (ALCLs-CT) and 28 Hodgkin-related (ALCLs-HR). T-cell phenotype was most common (58%), while B-cell, null and hybrid forms accounted for 27%, 13% and 2%. Clinically, ALCLs CT and HR differed as to mean age (27 v 34·3 years) and presentation; all ALCLs-HR showed mediastinal involvement, with bulky disease in 57%, and more frequent occurrence in stage II. In contrast, ALCLs-CT showed mediastinal masses in 58·5%, infrequently revealed bulky disease (24%), and were not specifically associated to stage. Among the ALCLs-CT, 68·4% achieved complete remission (CR), 24·4% partial remission (PR), one (2·4%) was resistant to therapy, and two (4·8%) had fatal drug toxicity. Of the ALCLs-HR, 67·8% reached CR, 14·3% PR, and 17·9% did not respond. In CR, ALCLs-CT showed a greater tendency to relapse (32·1% v 14·2%). At present, 65·8% of ALCLs-CT and 67·8% of ALCLs-HR are alive with overall survival/disease-free survival averages of 31/27 and 29/24 months respectively. Our data emphasize that, independently of subtype, ALCLs benefit from the application of third-generation protocols for high-grade non-Hodgkin's lymphomas.