原发性皮肤CD30~+间变大细胞淋巴瘤1例

患者男,41岁。双侧颈部、腋下及会阴区皮肤多发红色丘疹1月余。皮损组织病理示:皮肤原发性CD30~+大细胞淋巴瘤。免疫组化:CD30(+)、CD3(弱+)、CD4(+)、CD20(-)、CD79a(-)、CD56(-)、BCL-2(-)、S-100(-)、cyclind-1(-)、Ki67(+)阳性细胞数75%。诊断:皮肤原发性CD30~+大细胞淋巴瘤。多线化疗后病情进展,应用ICE作为解救方案,取得明显效果,疗效可达Cru,值得临床借鉴。     

原发性皮肤弥漫性大B细胞淋巴瘤,腿型

报告1例原发性皮肤弥漫性大B细胞淋巴瘤,腿型。患者男,77岁。双小腿出现多个红褐色斑块和结节半年。皮损组织病理检查:肿瘤细胞无嗜表皮现象,表皮与真皮之间可见一条无细胞的透明带,真皮内可见较大的异形淋巴细胞样细胞浸润,肿瘤细胞体积大,大部分呈明显的圆形,细胞核大异形性明显,核分裂象易见。免疫组化:CD20(+)、CD79α(+)、B细胞淋巴瘤(B-cell lymphoma,Bcl)-2(+)、B细胞特异性激活蛋白(Pax)-5(+)、多发性骨髓瘤癌基因1(multiple myeloma oncogene 1,MUM1)(+)、肌酸激酶(CK)(-)、CD3(-)、Bcl-6(-)、CD10(-)、细胞周期素d1(Cyclin D-1)(-)、黑色素A(Melan A)(-)、CD7(-)、生长因子受体结合蛋白(Gr B)(-)、CD30(-)、CD56(-)、CD4(-)、CD8(-)及细胞核增殖抗原(Ki-67)约80%(+)。诊断:原发性皮肤弥漫性大B细胞性淋巴瘤,腿型。经过R-CHOP方案(环磷酰胺+表柔比星+长春新碱+泼尼松)化疗后,肿块明显退缩。     

原发性皮肤弥漫大B细胞淋巴瘤(腿型)

报告1例原发性皮肤弥漫大B细胞淋巴瘤(腿型)。患者男,78岁。左小腿多个暗红色斑块、结节2个月余。左下肢皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,肿瘤细胞体积大,胞质淡染,核大、不规则,异形性明显,有丝分裂象易见。免疫组化:CD3(-)、CD8(-)、CD20(+)、CD79α(+)、B细胞淋巴瘤(Bcl)-2(+)、Bcl-6(+)、多发性骨髓瘤癌基因(MUM)-1(-)、CD10(-)、T细胞内抗原(TIA)(-),Kappa(k)(+)、Lambda(λ)(-)、增殖核抗原(Ki-67)约95%(+)。诊断:原发性皮肤弥漫大B细胞淋巴瘤(腿型)。治疗上给予R-CDOP方案[利妥昔单抗+(环磷酰胺+脂质体多柔比星+长春新碱+泼尼松)]化疗后,左小腿肿块缩小。     

播散型皮肤假性淋巴瘤

报告1例播散型皮肤假性淋巴瘤.患者男,48岁.因躯干及头面部丘疹、结节伴瘙痒3年余就诊.皮损组织病理检查:表皮基本正常,真皮内见致密结节状、以淋巴细胞为主的浸润,还可见嗜酸性粒细胞、浆细胞及组织细胞,淋巴细胞形状大小较为一致,无非典型性核,病变以真皮上半部为主,皮下组织未见异常.免疫组化检查:部分细胞CD4(++),CD8(++),CD3(++),CD45Ro(++),细胞毒颗粒相关蛋白(TIA)-1(+),穿孔素(perforin)(-),CD56(-);少数细胞CD20(++),CD79a(++);S-100蛋白部分(+),CD1a部分(+)提示朗格汉斯细胞增生,特殊染色示:结核杆菌及麻风杆菌抗酸染色均(-).诊断:播散型皮肤假性淋巴瘤(以T细胞增生为主).     

经典型卡波西肉瘤1例

报告1例经典型卡波西肉瘤。患者男,82岁,左下肢紫蓝色丘疹、结节1年,无特殊不适。近1个月双下肢水肿伴低热。皮肤专科检查:左侧下肢、左足、左手部可见多发性紫蓝色丘疹、结节,直径1～10 mm,触之较软,右侧肢体未受累。皮损组织病理:表皮角化过度,真皮内见大量毛细血管增生及梭形细胞增生,梭形细胞增生呈结节状,新生血管不同程度扩张、充血,形成裂隙样血管腔。免疫组化染色:HHV-8(+)、CD34(+)、CD31(+)、Vimetin(+)、D2-40(+)、Ki-67(+)约60%、CK (-)、DES(-)。结合临床表现、组织病理及免疫组化,诊断为经典型卡波西肉瘤。     

以肛周疣状增生物为首发症状的朗格汉斯细胞组织细胞增生症

报告1例以肛周疣状增生物为首发症状的朗格汉斯细胞组织细胞增生症。患儿男,2岁5个月,因肛周皮损1年就诊。皮肤科检查:头皮、耳后及躯干较多针尖至米粒大暗红色丘疹及瘀点;肛周见疣状增生物,部分糜烂。躯干皮损组织病理检查:真皮大量单个核细胞浸润,胞核椭圆或马蹄形,胞质丰富淡染,未见明显病理核分裂象,散在淋巴细胞浸润。免疫组化:CD1a、S-100蛋白及Langerin均(+)。肛周皮损组织病理检查:表皮角化过度,颗粒层存在,棘层肥厚,真皮浅层可见大量单个核细胞浸润,胞质丰富红染,核椭圆形或肾形,部分可见核沟,并可见嗜酸性粒细胞及淋巴细胞浸润。免疫组化示S-100蛋白(+)、CD1a(+)、CD68少量(+)及Langerin(+)。诊断:朗格汉斯细胞组织细胞增生症(皮肤和肝脏)。予JLSG-96方案诱导化疗一个疗程后,皮损明显消退。     

原发性皮肤间变性大细胞淋巴瘤1例

患者男,59岁。下颌反复出现红斑2年余。皮损病理组织示:角化过度,角化不全,表皮增生,表皮基底部细胞向下增生,细胞排列轻度紊乱,可见异常核分裂相,真皮乳头血管增生扩张,浅深层可见单一核细胞浸润,细胞体积大,核异型性明显。免疫组化染色结果显示:CD3(+)、CD4(+)、CD8(+)、CD20(+)、CD79a(+)、CD30(+)、S-100(-)、CK20(-)、HCK(-)、Ki-67阳性细胞约50%~60%。诊断为原发性皮肤间变性大细胞淋巴瘤。     

原发皮肤的儿童结外NK/T细胞淋巴瘤-鼻外型1例及文献复习

目的报道1例原发皮肤的儿童结外NK/T细胞淋巴瘤,鼻外型,并回顾文献,学习该病的临床特征、组织病理、免疫组化及治疗预后特点,以提高临床医生对该病的认识。方法分析本例原发皮肤的儿童结外NK/T细胞淋巴瘤-鼻外型患者的临床表现、辅助检查、病理组化及治疗预后,并复习近年国内外相关文献。结果 11岁男性患儿表现为皮肤结节、斑块伴乏力,肝脾淋巴结明显肿大。实验室检查示白细胞和血小板降低,肝酶升高,乳酸脱氢酶升高,凝血时间延长,血液EBV-PCR高度复制。组织病理提示局部或弥漫大小不一淋巴细胞浸润,可见核碎裂,部分细胞核大深染。免疫组化:CD3(+)、CD20(-)、CD56(+++)、颗粒酶B(+)、T细胞胞浆内抗原-1(+)、Ki-67约30%~40%(+);EB病毒编码RNA原位杂交(+++)。诊断:原发皮肤的结外NK/T细胞淋巴瘤,鼻外型。告知病情后家属放弃治疗。结论不同于鼻型,本病的临床表现特异性不高,易误诊为脂膜炎等,多合并系统受累,确诊依靠组织病理、免疫组化、EBV病原学检验及结合临床表现。尽管采用强势化疗,本病仍预后不良。     

Sézary综合征1例

患者男,86岁,全身反复红斑、脱屑伴瘙痒2年。2年前患者全身弥漫性红斑伴瘙痒,多次治疗病情反复。1周前右侧腰部出现一鸡蛋大小结节,红肿热痛。腹部皮肤病理见表皮内真皮浅层血管周围广泛淋巴样细胞浸润,有Pautrier假脓肿形成;免疫组化见淋巴样细胞为CD3ε(+)、CD4(+)、CD8(-)。淋巴结病理活检见淋巴结结构破坏,大量异型的淋巴细胞弥漫增生;免疫组化见淋巴样细胞为CD3ε(+)、CD5(+)、CD4(+)、CD8(-)。外周血涂片:查见Sézary细胞。TCR-γ基因重排:提示有克隆性扩增峰。诊断:Sézary综合征。放弃治疗,3个月后死亡。     

母细胞性浆细胞样树突细胞肿瘤

报告1例母细胞性浆细胞样树突细胞肿瘤。患者女,26岁。面部结节、斑块半年。皮肤科检查:右颧部可见约4 cm×6 cm暗红色浸润性斑块,类圆形分叶状,境界清楚,皮损中央破溃。皮损组织病理检查:真皮全层及皮下脂肪间见较多致密的异形淋巴细胞浸润,细胞中等大小,核膜薄,染色质细,核分裂象易见。免疫组化:CD4(+)、CD56(+)、CD123(+)、CD43(+)、CD34(+),Ki-67(60%阳性);CD20、CD79a、丝氨酸蛋白酶(granzyme)B、CD3、CD45RO、CD68、末端脱氧核苷酸转移酶(TDT)、髓过氧化物酶(MPO)、T细胞内抗原(TIA)-1均阴性。诊断:母细胞性浆细胞样树突细胞肿瘤。     

生存素、Ki67在皮肤结外鼻型NK/T细胞淋巴瘤中的表达及其意义

目的 探讨生存素、Ki67在皮肤结外鼻型NK/T细胞淋巴瘤中的表达及意义。方法 选取确诊的以皮损为首发表现的15例皮肤结外鼻型NK/T细胞淋巴瘤为研究对象,常规SP免疫组化染色法检测生存素、Ki67。每张切片选取5个有代表性的高倍镜视野,每个视野随机计数200个肿瘤细胞,分别计算1000个肿瘤细胞内生存素、Ki67阳性细胞百分比。结果 15例皮肤结外鼻型NK/T细胞淋巴瘤中,9例瘤细胞表达CD56,13例表达CD3ε,15例表达TIA-1,10例表达粒酶B,2例表达CD3。15例均表达1 ～ 2个T细胞抗原（CD2、CD45RO或CD7）,1例表达βF1,3例表达CD30的病例阳性细胞均为大瘤细胞。所有病例均不表达CD4、CD5、CD8、CD20和CD79α。14例中3例被检出TCR-γ基因克隆性重排,15例EBER1/2原位杂交均阳性。15例中,11例（73.3%）表达生存素,阳性细胞表达率为23.97% ± 18.35%;14例（15例中1例掉片）均表达Ki67,阳性细胞表达率41.20% ± 19.52%。核分裂0 ～ 2个/高倍视野的9例Ki67表达阳性细胞率为25.27% ± 12.96%,核分裂 ＞ 2个/高倍视野的6例为58.23% ± 16.02%,两组差异有统计学意义（F = 19.14,P = 0.001）。皮肤结外鼻型NK/T细胞淋巴瘤中生存素、Ki67都有较高的表达,生存素与Ki67表达之间无相关性。结论 生存素、Ki67的高表达可能在皮肤结外鼻型NK/T细胞淋巴瘤发生发展中起着一定作用。     

皮肤的结外鼻型NK/T细胞淋巴瘤1例及文献复习

目的报道1例皮肤结外鼻型NK/T细胞淋巴瘤,以引起临床和病理医师对此病的关注。方法通过临床病理分析结合免疫组化染色、EB病毒原位杂交及T细胞受体基因重排的PCR检测分析确诊。结果左膝内后方皮损初次活检诊断为结节性脂膜炎,1个月内再次活检示真皮和皮下脂肪内肿瘤大片坏死,瘤细胞异型性明显,血管中心性浸润和血管坏死。瘤细胞表达CD2,CD8,CD45RO,CD56,TIA-1,GranzymeB和LMP-1,EB病毒(+);未检测到TCR-γ的克隆性基因重排。诊断为皮肤的结外鼻型NK/T细胞淋巴瘤。结论皮肤的结外鼻型NK/T细胞淋巴瘤恶性度高、易误诊、预后差;诊断有赖于常规组织病理结合分子病理技术。     

Cutaneous peripheral T-cell lymphoma of cytotoxic phenotype mimicking extranodal NK/T-cell lymphoma

Cutaneous peripheral T-cell lymphoma unspecified is a rare neoplasm that is infrequently associated with Epstein-Barr virus (EBV) infection. In contrast, extranodal natural killer (NK)/T-cell lymphoma, although also rare, is known to be strongly associated with EBV and occurs most commonly in the nasal region. We report the case of a 55-year-old male who presented with fever and an indurated cutaneous plaque with ulceration. This cutaneous neoplasm showed diffuse dermal lymphomatous infiltration and tumor necrosis, with neoplastic cells expressing CD2, cytoplasmic CD3 (CD3ε), CD8, CD16, CD30, T-cell intracellular antigen-1, and granzyme B but not CD56, BF1, or T-cell receptor (TCR) δ1. Furthermore, the tumor cells were noted to be diffusely positive for EBV by in situ hybridization. A monoclonal TCR gene rearrangement was demonstrated. The disease showed an aggressive clinical course, and the patient died within 3 weeks of diagnosis without complete staging or chemotherapy. According to the 2005 World Health Organization/European Organization for Research and Treatment of Cancer scheme for cutaneous lymphoma and the 2008 WHO classification for lymphoid neoplasms, our case would have been classified as a nasal type extranodal NK/T-cell lymphoma with T-cell lineage. However, the expressions of CD8 and CD16, in addition to a monoclonal TCR gene rearrangement, are unusual findings in NK/T-cell lymphoma, and we believe such a phenotype/genotype should be more appropriately classified as an EBV-positive peripheral T-cell lymphoma, unspecified with a cytotoxic phenotype. Detailed clinicopathologic and molecular studies of similar cases may shed light on the prognostic impact of NK vs. T-cell lineage on extranodal NK/T-cell lymphomas.     

Rearranged T-cell receptor gene and positive Epstein-Barr virus-encoded nuclear RNA in an extranodal NK/T-cell lymphoma with cutaneous manifestation only: case study

Natural killer (NK)/cytotoxic T-cell lymphoma, a new type of cutaneous neoplasm, has been described recently in the World Health Organization/European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. We report an 11-year-old boy who had had erythematous plaques and blisters on his face and hands for 4 years and infiltrating plaques and necrosis on his extremities for 4 months. Routine clinical and laboratory examinations found no primary nasal involvement. Biopsies taken from nasal mucosa and skin showed that the tumour only involved dermis and subcutaneous tissue, and the infiltrated lymphohistiocytic tumour cells were CD56+, TIA+, CD45RO+ and CD30+. In situ hybridization for EBV-encoded nuclear RNA was positive. Clonal T-cell receptor-gamma2 gene rearrangement was positive. A diagnosis of extranodal NK/T-cell lymphoma, nasal type, was made. This is a rare case, with slow course and survival for >51 months with the presentation only occurring in the skin.     

Nasal‐type extranodal natural killer/T‐cell lymphoma presenting with extensive leg ulcers

Primary cutaneous T‐cell lymphomas are rare and can be difficult to classify precisely. We present a case of extranodal natural killer (NK)/T‐cell lymphoma in a previously healthy, immunocompetent man who presented with extensive necrotic leg ulcers and disseminated skin nodules. Immunohistochemical studies revealed that the tumour cells were positive for CD3, CD30, granzyme B and T‐cell intracellular antigen‐1, and negative for CD5 and CD56, with positive staining for Epstein–Barr virus (EBV) RNA on in situ hybridization. A diagnosis of extranodal NK/T‐cell lymphoma was made, based on the presence of cytotoxic granules and positive EBV RNA staining. The patient was treated with a regimen of chemotherapy comprising corticosteroids, intravenous methotrexate, ifosphamide, L‐asparginase and etoposide with initial response.     

Primary cutaneous CD56 positive lymphoma: a diagnostic conundrum in an unusual case of lymphoma

We present an unusual case of a CD56-positive T-cell lymphoma exhibiting immunophenotypic characteristics of both γδ T-cell lymphoma and extranodal NK/T-cell lymphoma, nasal-type. The patient presented with a 2-month history of rapidly progressive, pruritic and cutaneous nodules on his arms. A biopsy showed a dense pan-dermal infiltrate of markedly atypical CD3-positive lymphocytes, compatible with tumor stage cutaneous T-cell lymphoma. Retrospective review of a preceding biopsy and flow cytometric analysis, performed at an outside institution, showed strong expression of surface CD3, CD7, CD43 and γδ T-cell receptor (TCR), findings consistent with a diagnosis of cutaneous γδ T-cell lymphoma. In light of these data, we performed additional studies that showed diffuse positive staining of the atypical lymphocytes for CD56, CD4 and CD43 as well as Epstein-Barr virus-encoded small nonpolyadenylated RNA (EBER). Interestingly, this case displays characteristic features of γδ T-cell lymphoma, with strong surface expression of CD3 and γδ-TCR, as well as characteristics of natural killer (NK)/T-cell lymphoma, including expression of CD4 and EBER positivity, that represent two separate categories in the current classification of cutaneous lymphomas. Taken together, these findings underscore the difficulty of rendering an unambiguous classification of the presented neoplasm given the close ontogenetic relationship between NK and cytotoxic T-cells and highlight the need for continued reevaluation of the current classification system.     

Cutaneous natural killer/T-cell lymphoma

Lymphomas are classified as either Hodgkin's or non-Hodgkin's. The 2 subtypes of non-Hodgkin's lymphoma that can present primarily in the skin are cutaneous T-cell lymphoma and cutaneous B-cell lymphoma, both of which tend to be low-grade malignant neoplasms. Recently another distinct subtype of lymphoma was discovered, the natural killer (NK)/T-cell lymphoma, which can involve the skin in a primary or secondary fashion. The NK/T-cell subtype of lymphoma is characterized by the expression of the NK-cell antigen CD56. These CD56(+) lymphomas are further subdivided into nasal NK/T-cell lymphomas that commonly present as midfacial destructive disease and non-nasal NK/T-cell lymphomas that often arise in extranodal locations, including the skin. We report a case of aggressive NK-cell leukemia/lymphoma with numerous secondary cutaneous lesions and review the clinical and histopathologic spectrum of non-nasal CD56(+) lymphomas, with an emphasis on the dermatologic findings.     

累及皮肤的结外鼻型NK/T细胞淋巴瘤

报告1例累及皮肤的结外鼻型NK/T细胞淋巴瘤.患者男,72岁.确诊结外鼻型NK/T细胞淋巴瘤8年后,鼻根部出现红肿、破溃.根据皮损、组织病理检查和免疫组化染色结果确诊.     

36例皮肤天然杀伤细胞/T细胞淋巴瘤临床病理学研究

目的 探讨皮肤天然杀伤细胞(NK)/T细胞淋巴瘤临床病理学特点、与EB病毒的关系及预后。方法 收集2000—2010年北京大学医学部病理学系确诊为皮肤NK/T细胞淋巴瘤36例,分为原发与继发两组,分别观察临床病理学特点及与EB病毒的关系,并进行随访。结果 36例皮肤NK/T细胞淋巴瘤中,原发13例,继发20例,未能明确原发或继发3例。原发性与继发性皮肤NK/T细胞淋巴瘤均以男性好发,但两组男女性别比差异无统计学意义(P＞0.05)。与原发者相比,继发者发病年龄早(中位年龄,43.5比54岁,P＜ 0.05)、且临床上出现B症状(包括发热、盗汗或体质量下降)及多发皮损改变的频率较高(P值分别为＜0.05和＜0.01)。EB病毒在原发和继发病例中的检出率类似,分别为92.3％和85％。36例皮肤NK/T细胞淋巴瘤中位生存期为8个月,其中继发性皮肤NK/T细胞淋巴瘤中位生存期为6个月,明显短于原发者(18个月,x2= 6.074,P＜0.05)。结论 皮肤NK/T细胞淋巴瘤是一组与EB病毒密切相关、临床侵袭性强的肿瘤。但原发者较继发者发病年龄晚、预后较好。