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。     

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。     

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。     

睾丸间变性大细胞T细胞性淋巴瘤一例

患者男 ,42岁。右侧睾丸肿大 6个月伴坠胀痛 2个月 ,于 2 0 0 1年 11月 12日入院行睾丸切除术。术前检查 :全身浅表淋巴结及肝脾均未触及 ,Ｘ线胸片及腹部Ｂ超检查均无异常 ,右侧睾丸 9 0ｃｍ× 5 5ｃｍ× 4 5ｃｍ大小 ,透光试验 ( - ) ,血象正常。病理检查 :睾丸连同附睾精索 8ｃｍ× 5ｃｍ× 4ｃｍ大小 ,切面睾丸组织被肿瘤组织代替 ,灰白灰红 ,质软细嫩 ,鱼肉状 ,伴点状坏死 ,累及附睾组织。镜下观察 :瘤细胞弥漫分布 ,睾丸结构被破坏 ,累及白膜并浸润至附睾组织内 ,伴多灶性坏死 ,仅见个别萎缩退变的曲细精管。瘤细胞多形性明显 ,大小…     

原发性淋巴结外间变性大细胞淋巴瘤临床病理分析

目的探讨原发性淋巴结外间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的病理形态、免疫表型特征及预后特点。方法对29例原发性淋巴结外ALCL进行形态学观察、免疫组化标记及随访,并结合相关文献进行讨论。结果本组淋巴结外ALCL共29例,男女之比为1.6∶1,平均年龄46.2岁。发生于皮肤12例、消化道7例、骨5例、口鼻黏膜3例、肺及乳腺各1例。29例患者中随访12例,随访时间3～45个月。29例均经外科手术切除局部病变或脏器,大部分再辅以化疗和放疗。12例随访病例中,手术切除病变后接受单纯化疗7例,放化疗者2例,未经放化疗者3例。其中8例死亡。ALCL组织学形态多样。免疫组化:29例ALCL均表达CD30,大多数表达CD3和(或)CD43(26/29),部分表达EMA、ALK-1、GranB和Perforin;不表达CKpan、CD20、CD79α、HMB45、CD68、CD15和CD117。结论发生在淋巴结外的ALCL并非罕见,临床表现无特异性,诊断依赖于组织病理学及免疫组化标记,淋巴结外(除外皮肤)的ALCL预后相对较差。该病应与弥漫性大B细胞淋巴瘤、霍奇金淋巴瘤...     

原发性皮肤间变性大细胞淋巴瘤临床病理分析

目的 探讨原发性皮肤间变性大细胞淋巴瘤(C-ALCL)的临床病理特征、免疫表型及预后.方法 分析8例C-ALCL的临床病理资料,复习HE切片,进行T淋巴细胞、B淋巴细胞、活化淋巴细胞和细胞毒性等16种标记的免疫组织化学染色,原位杂交检测EB病毒.结果 8例中男3例,女5例,中位年龄49.5岁.临床上以皮肤无症状的单个红色结节、肿块为主要表现,组织学上肿瘤细胞在真皮与皮下脂肪内大片状、弥漫性浸润.瘤细胞以大细胞为主,异形性明显.8例C-ALCL的瘤细胞CD30阳性细胞数均大于75%.瘤细胞均表达1～3个T细胞标记(CD3、CD5或CD45RO)及1～3个细胞毒性标记[T细胞内抗原(TIA)-1、颗粒酶B或穿孔素].表达白细胞共同抗原(LCA)为8例、CIM为5例、CD8为1例、间变性淋巴瘤激酶(ALK)-1为1例、上皮细胞膜抗原(EMA)为3例,均不表达CD15、CD20、CK和HMB45.EBER 1/2原位杂交均为阴性.获随访的6例中5例存活,1例死亡(死因不详).结论 C-ALCL有独特的临床病理表现和免疫表型,预后较好.EB病毒与C-ALCL可能无明确的相关性.     

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。     

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。     

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。     

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。     

75例原发性胃肠道淋巴瘤的临床病理分析

目的:探讨原发性胃肠道淋巴瘤(primary gastrointestinal lymphoma,PGIL)的临床特点、诊断、病理特征和治疗.方法:收集2011年1月至2017年1月就诊于武汉大学人民医院75例PGIL患者的临床及病理资料,并进行回顾性分析.结果:75例PGIL患者的男女性别比为1.5:1,年龄(56.96±14.51)岁,其中43例(57.33％)患者以腹部隐痛为主要症状.51例(68.00％)患者CEA升高,17例(32.69％)患者行大便隐血试验(+).胃镜/肠镜诊断阳性率为74.58％.9例直肠淋巴瘤患者之中,有5例直肠指诊阳性,直肠指诊的阳性率为55.56％.75例PGIL患者均已行病理检查,45例弥漫性大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL),13例黏膜组织相关淋巴瘤黏膜组织相关淋巴瘤(mucosa-associated lymphoid tissue,MALT),6例间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL),4例外周T细胞淋巴瘤(peripheral T-cell lymphoma,PTCL),7例为其它病理类型.按照Ann Arbor标准分期,ⅠE期7例(9.33％),ⅡE期25例(33.33％,ⅢE 11例(14.67％),IVE期32例(42.67％).41例患者行CHOP化疗方案,12例患者行R-CHOP化疗方案,3例行手术治疗,19例患者放弃治疗.结论:PGIL好发于中老年男性,临床症状以腹痛为主,确诊主要依靠内镜下活检,内镜下诊断不明确的患者可行腹腔探查术明确诊断.CEA、大便隐血试验对PGIL的辅助诊断有一定价值,而直肠指诊对直肠淋巴瘤的初诊有重要意义.早期PGIL患者以PGL多见,而中晚期PGIL患者以PIL多见.病理类型以DLBCL和MALT为主.DLBCL型PGIL治疗首选CHOP/R-CHOP,MALT型PGIL患者首选HP根除疗法,手术适用于PGIL的并发症治疗.     

原发心脏弥漫大B细胞淋巴瘤5例临床病理分析

目的 探讨原发心脏弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)的临床病理特点、诊断、治疗及预后.方法 回顾性分析5例原发心脏DLBCL的临床病理资料,分别行HE染色、免疫组化EnVision两步法染色,应用原位杂交以及荧光原位杂交(fluorescence in situ...     

Clusterin is widely expressed in systemic anaplastic large cell lymphoma but fails to differentiate primary from secondary cutaneous anaplastic large cell lymphoma

A recent study by Wellmann et al (Blood. 2000;96:398-404) detected clusterin expression in all 36 systemic anaplastic large cell lymphomas (ALCLs) tested, but not in any of 9 primary cutaneous ALCLs. Our purpose was to confirm the diagnostic usefulness of clusterin in systemic ALCL and to evaluate its efficacy in distinguishing primary cutaneous ALCL from secondary skin involvement by systemic ALCL. We examined clusterin expression by paraffin immunohistochemical analysis in 41 systemic ALCLs (18 ALK-1+ and 23 ALK-1-), 9 primary cutaneous ALCLs, and 4 secondary cutaneous ALCLs. Clusterin was positive in 95% of systemic ALCLs (39/41), including 100% (18/18) of the ALK-1+ cases and 91% (21/23) of the ALK-1- cases. Five (56%) of 9 primary and 3 (75%) of 4 secondary cutaneous ALCLs were positive for clusterin. Our observations confirm the diagnostic usefulness of clusterin in systemic ALCL, especially in the ALK-1- cases. However, our data fail to demonstrate its value in distinguishing primary from secondary cutaneous ALCL.     

Association of expression of CD44v6 with systemic anaplastic large cell lymphoma: comparison with primary cutaneous anaplastic large cell lymphoma

CD44 is a ubiquitous multifunctional cell surface adhesion molecule family. High expression of the standard form, CD44s (CD44), and its variant form, CD44v6, has been reported to be associated with tumor dissemination in non-Hodgkin lymphoma. To evaluate the potential role of CD44 and/or CD44v6 in different entities of anaplastic large cell lymphoma (ALCL), 30 cases of systemic ALCL (sALCL; 20 cases) and primary cutaneous ALCL (cALCL; 10 cases) were compared for expression of CD44 and CD44v6 by immunohistochemical staining. Expression of CD44v6 also was analyzed with respect to expression of anaplastic lymphoma kinase (ALK) in sALCL. No difference of CD44 expression was noted between sALCL and cALCL In contrast, expression of CD44v6 was found in 18 (90%) of sALCL cases and in 5 (50%) of cALCL cases. There was no correlation between expression of CD44v6 and expression of ALK in sALCL. These results indicate that expression of CD44v6 rather than CD44 correlates with sALCL. Furthermore, these results suggest that CD44v6 and ALK may be independent predictors of risk for the systemic phenotype of ALCL.     

ALK-positive anaplastic large cell lymphoma with primary bone involvement in children

We describe the clinical, radiologic, and pathologic features of primary bone anaplastic large cell lymphoma (ALCL) in 3 boys. Radiologic imaging showed lytic lesions involving sacrum, femur, or rib. Bone was the only site of disease in 2 cases; an associated partial lymph node was involved in case 3. Differential diagnoses included osteomyelitis and small round cell tumors of childhood, particularly Ewing sarcoma. Preoperatively, ALCL was not a diagnostic consideration in any case. Two cases showed classic large pleomorphic cells; 1 showed a composite pattern with a distinct small cell component and the more typical large cell type. Neoplastic cells in all cases showed strong CD30 and anaplastic lymphoma kinase expression with relatively weak epithelial membrane antigen positivity. Cytotoxic granule protein was expressed in 2 cases. All cases showed unusually strong expression of neuron-specific enolase (NSE). Two patients were disease-free at last follow-up (15 months and 11 years); 1 patient died of disseminated disease within a year of diagnosis. ALCL should be considered a diagnostic possibility when evaluating neoplastic bone lesions in children. Although expression of NSE in ALCL has not been emphasized in the literature, it is worth noting because it may pose a diagnostic pitfall.     

Leukemic phase of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma

Anaplastic large cell lymphoma (ALCL) is a distinct type of CD30+ T/null-cell non-Hodgkin's lymphoma that frequently involves nodal and extranodal sites. The presence of leukemic phase in ALCL is extremely rare and occurs exclusively with ALK1-positive ALCL. We describe two patients with ALK1-positive ALCL who developed a leukemic phase with rapid progression of the disease. Immunophenotypic pattern assessed on peripheral blood by flow cytometry revealed CD45, CD30, and CD25 positivity in both cases but NPM-ALK1 was expressed in only one case. Both patients developed leukemic phase as a terminal event of the disease and we share the immunophenotypic features of both cases.