结外皮肤NK/T细胞淋巴瘤一例

患者，男，77岁。全身散发皮肤肿块1个月。组织病理示：表皮变薄，真皮全层大量淋巴样细胞弥漫浸润，部分区域具有亲表皮性，部分细胞胞体大,可见核分裂像。诊断：结外皮肤NK/T细胞淋巴瘤。患者确诊后转至肿瘤科，给予P-Gemox方案化疗。     

ALK+ CD30+原发性皮肤间变性大细胞淋巴瘤一例

患者男,27岁.左侧腹股沟包块2个月,间断发热伴疼痛3周.皮损初起于左腹股沟内侧皮肤,为暗红色丘疹,后增大破溃,伴左腹股沟淋巴结肿大、发热.体检:左腹股沟内侧可触及直径约4 cm浸润性肿块,中央约1 cm溃疡,少许血性浆液渗出,明显触痛,皮损外侧可触及数个蚕豆大小肿大淋巴结,质硬,粘连呈条索状,活动度差.皮损组织病理检查:真皮深层至皮下脂肪层致密片状异形淋巴样细胞浸润,淋巴结穿刺部分区域见团片状异形淋巴样细胞.浸润细胞免疫组化标志示:CD30、CD43、CD45阳性,ALK表达为核浆型,诊断为ALK+ CD30+原发性皮肤间变性大细胞淋巴瘤.治疗:患者接受3次环磷酰胺+表阿霉素+长春新碱+醋酸泼尼松化疗(CEOP)后,皮疹完全消退,破溃处愈合.     

原发性皮肤CD_(30)~+间变性大细胞淋巴瘤1例

患者男,71岁。右上肢反复出现结节2年。右肘部外侧密集数十个蚕豆至核桃大结节,部分表面破溃。右上胸部有片状浸润性斑块、结节。右肘部结节组织病理示:真皮全层及皮下脂肪均可见肿瘤细胞呈弥漫、致密浸润性生长,无亲表皮现象,肿瘤细胞为淋巴样细胞,体积较大,形态多样,明显异型性。免疫组化示:肿瘤细胞CD30强阳性,Ki67细胞增殖指数约80%,CD43阳性,LCA,CD45RO,CD3部分阳性,ALK,EMA,CD15,CD10,CD20,CK-P,S-100,HMB45,Vimentin,TDT及CD68均阴性。诊断:原发性皮肤CD3+0间变性大细胞淋巴瘤(PC-ALCL)。采用COP方案化疗一个周期,2个月后皮损全部复发,死亡。     

牛痘样水疱病样皮肤NK/T细胞淋巴瘤一例

患儿男，11岁，面部、手臂出现红斑、水疱4年，四肢肿块、溃疡4个月。逐渐感觉乏力，有不规则间歇发热。体检：体温37.7 ℃。全身淋巴结无明显肿大，口腔，颜面和鼻部皮肤片样轻度浸润的粉红色斑片，四肢见大小不等的暗红色浸润性结节和肿块，结节直径2.0 ~ 18 cm不等，部分表面有坏死和黑色结痂，较大的结节表面形成溃疡，坏死性溃疡深达肌肉并有白色脓性分泌物。皮损边界清楚，质地较硬，有压痛，部分结节周围见到卫星样小结节。取自大腿的皮损病理检查发现，表皮真皮全层至皮下可见大量形态不一、中等大小的肿瘤细胞浸润，肿瘤细胞胞质清晰，核大，扭曲状，染色质丰富，细胞呈巢或散在，可见围管样分布；真皮和皮下见碎核和反应性组织细胞浸润，肿瘤细胞浸入毛囊和外泌汗腺。免疫组化：胞质CD3在核周区可见阳性染色，CD56、CD45RO和TIA-1强阳性（+++），部分肿瘤细胞CD30、CD8和Ki 67阳性（++）。EB病毒编码小核RNA（EBER）阳性，TCR-γ 基因重排阳性。最后诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤，这是1例罕见的原发于皮肤长达51个月的进展缓慢的病例。     

原发性皮肤弥漫大B细胞淋巴瘤（腿型）一例

报告1例原发性皮肤弥漫性大B细胞淋巴瘤（腿型）。患者男,51岁。右小腿出现多个红色结节5个月,组织病理检查：表皮和真皮之间见无细胞浸润带,真皮内淋巴样细胞浸润,细胞体积大,异形。肿瘤细胞CD20（++）,Bcl-6(+),Bcl-2(+),Pax-5(+),Ki-67 50％-75％(+),CD79a(+),MUM-1（+）,CD10（-）,诊断为原发性皮肤弥漫性大B细胞淋巴瘤（腿型）,给予CD20单克隆抗体加CHOP方案治疗,病情好转。     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤1例临床病理观察并文献复习

报告1例原发性皮肤CD4 多形性小/中T细胞淋巴瘤.患者女,45岁.右膝右上方反复红斑、结节15年.组织病理检查示真皮全层及皮下脂肪层弥漫性结节性致密小到中等大淋巴样细胞浸润.细胞有异形,其间混杂少量炎性细胞,无亲表皮现象.免疫组化检查示全T抗原缺失的Th表型.诊断:原发性皮肤CD4 多形性小/中T细胞淋巴瘤.     

CD8阳性皮肤γ/δ T细胞淋巴瘤一例

【摘要】 患者女,44岁。躯干多发斑块、溃疡2个月。皮损初起为红色斑块,迅速破溃形成溃疡。皮肤组织病理检查示真皮全层及皮下脂肪层弥漫中等至较大的淋巴样细胞浸润,细胞明显异形性,亲表皮生长。免疫组化结果显示,异形细胞表达CD3、CD8、CD56、颗粒酶B（GranB）、穿孔素,而TCRα/β、CD4、CD5、CD20和CD79α不表达。EB病毒（EBV）检查阴性。T细胞受体基因重排示γ基因重排阳性。诊断：皮肤γ/δ T细胞淋巴瘤。患者放弃治疗,于发病20个月后死亡。     

皮肤CD8+亲表皮性T细胞淋巴瘤一例

75岁男性患者,躯干、四肢出现瘙痒性红斑、斑块和苔藓样皮疹5年.皮肤科检查:颈部、躯干和四肢泛发暗红、灰黑色浸润性斑片和斑块,以背部为甚.全身浅表淋巴结无肿大,未发现系统受累情况.组织病理显示:表皮不规则增生,棘层肥厚,表皮内可见核深染的异形淋巴细胞浸润,形成Pautrier微脓肿.真皮浅层有片状和团块状淋巴样细胞浸润,细胞有异形性.免疫组化标记:肿瘤细胞CD3、CD8、GrB、LCA和TIA-1阳性,CD4和CD5阴性.符合原发性皮肤CD8+亲表皮性T细胞淋巴瘤诊断.口服维胺酯治疗病情有所好转.     

以皮肤损害为首发表现的结外鼻型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,分析其临床表现、组织病理特点及治疗和预后,以提高皮肤科临床医生对本病的诊治水平.方法:通过临床表现、组织病理分析,结合免疫组化染色、EB病毒原位杂交确诊.结果:颈后皮损组织病理示真皮浅中层血管附属器周围几大灶淋巴样细胞浸润,细胞核大,胞浆透明,异型性明显.瘤细胞表达CD2、CD3、CD5、CD7、CD8、GranzymeB、Ki-67,而不表达CD56,EB病毒( + ).诊断为皮肤的结外鼻型NK/T细胞淋巴瘤.结论:结外鼻型NK/T细胞淋巴瘤具有独特的组织病理及免疫组化特征,恶性度高、易误诊、预后差.     

原发性皮肤弥漫大B细胞淋巴瘤1例

患者男,58岁。右颈肩部肿物7个月,加重3周。皮肤科情况:右颈肩部见20cm×20cm大小肿块,质地中等度硬,未触及疼痛。皮损组织病理示:真皮中部至皮下脂肪组织内见密集淋巴样细胞浸润,细胞体积较大,胞浆丰富,核圆形、卵圆形或不规则形,核膜厚,染色质粗颗粒状,核仁明显,核分裂可见。免疫组化显示:大淋巴样细胞CD20弥漫(+),CD79a,BCL6,MUM1(+),CD3,CD4,CD8散在(+),CD56,CD30,CD10,BCL-2(-),Ki67(+)约80%。诊断原发性皮肤弥漫大B细胞淋巴瘤。经8个疗程CHOPE方案化疗及1个月放疗,皮肤肿块完全消退。随访5年未见复发。     

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.     

原发性皮肤CD30阳性大细胞淋巴瘤1例

报告1例原发性皮肤CD30阳性大细胞淋巴瘤。患者女,65岁。左上臂出现结节并逐渐扩大6年,组织病理检查发现真皮弥漫性致密淋巴样细胞浸润,多数细胞核大、深染,可见病理分裂相。免疫组织化学染色结果示:CD30、LCA、CD4、CD45RO阳性,CD20、CD68、CD79a、TIA阴性。诊断为原发性皮肤CD30阳性大细胞淋巴瘤,行局部放疗后治愈,随访3年未复发。     

特发性免疫球蛋白缺乏症继发外周T细胞淋巴瘤一例

目的 报道1例特发性免疫球蛋白缺乏症继发外周T细胞淋巴瘤。方法 通过病史、临床及实验室检查,病理及免疫组化以排除其他肿瘤性疾病,并观察临床用药治疗效果。结果 25岁男性患者的皮损呈丘疹、斑块及结节.发生于左臀部、上唇、鼻翼等部位。主要表现为反复发生的严重感染.IgG明显低于正常水平(<0.069 g/L)。皮损组织病理检查见真皮内大量大小不等的淋巴细胞。呈弥漫性分布.核异形,有丝分裂明显。皮损免疫组化检查.CD7、CD2、CD4、LAT均阳性,Ki-1、CD20、OCT2、CD56、CD68、CD79a均阴性。结论 本患者的诊断主要依据相关的实验室检查.临床表现是本病诊断的重要线索.有助于疾病的早期诊断。     

种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤

报告1例大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤.患者男,80岁.全身水肿性红斑伴水疱、大疱,诊断为大疱性类天疱疮.在发病7个月后,右胫前出现多个暗紫红色结节,逐渐增多、增大,并扩展至右股内侧和背部.皮损组织病理检查示真皮全层及皮下脂肪小叶弥漫致密的淋巴细胞浸润,可见多数淋巴细胞体积增大,形态不规则,核大深染,可见核分裂相.免疫组化染色:CD20( ),bcl-2( ),bcl-6( ).诊断:原发性皮肤弥漫性大B细胞淋巴瘤.在肿瘤出现后1个月,患者大疱性类天疱疮的病情出现反复,通过CHOP方案治疗,病情明显好转.     

成人种痘样水疱病样淋巴瘤一例并文献复习

本文报道1例种痘样水疱病样淋巴瘤并对WHO最新分类、EBV感染相关发病机制、鉴别诊断及治疗方面的相关文献进行复习。患者,男,38岁。全身反复皮疹30年,加重1年,患者病程中伴有发热,皮损预后遗留凹陷性瘢痕。血清EB病毒抗体IgG阳性。皮损组织病理示：真皮全层及皮下脂肪小叶内大量淋巴细胞浸润。免疫组化染色示：CD2(+++),CD3(++),CD8(++),TIA-1(++),CD56(少量+),CD20(灶状+) ,Ki-67＞30%。EB病毒原位杂交：阳性。诊断：种痘样水疱病样淋巴瘤。     

Lymphoma with large-plaque parapsoriasis treated with PUVA

We report on a 78-year-old Japanese woman with a 50-year history of large-plaque parapsoriasis that had evolved into cutaneous T-cell lymphoma. Her large-plaque parapsoriasis had been treated with psoralen plus ultraviolet A for 10 years. Subsequently an isolated nodule appeared on her right lower leg. Prior or concurrent patches or plaques were absent. Histology revealed a diffuse nonepidermotropic infiltrate of large lymphocytes in the dermis, which had enlarged nuclei and prominent nucleoli. A diagnosis of CD30- cutaneous large T-cell lymphoma was made. Following systemic chemotherapy, there was clinical improvement. No evidence of recurrence or systemic lymphoma has subsequently been found.     

CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate

The CD4(+) CD56(+) hematodermic/plasmacytoid dendritic cell tumor is a rare, highly aggressive, systemic neoplasm for which effective therapies have not yet been established. These tumors express CD4, CD56, CD123, and T-cell leukemia/lymphoma (TCL)-1 and are clinically characterized by cutaneous involvement with spread to bone marrow and blood, and poor prognosis with current chemotherapy regimens. We describe a Caucasian woman who presented with plasmacytoid dendritic cell tumor, but an absence of systemic symptoms. Clinically, multiple cutaneous lesions were brown to violaceous firm nodules on the face, arms, and trunk. The patient underwent two courses of cyclophosphamide, Adriamycin, vincristine, and prednisone chemotherapy but relapsed quickly. The investigational agent, pralatrexate (30 mg/m(2)) was given weekly with vitamin B12 and folic acid and resulted in remarkable clinical response with regression of skin tumors. Our observation highlights pralatrexate as a promising therapeutic option for hematodermic/plasmacytoid dendritic cell lymphoma/leukemias.     

Lymphomatoid Papulosis Type D: A Newly Described Variant Easily Confused With Cutaneous Aggressive CD8-Positive Cytotoxic T-Cell Lymphoma

ABSTRACT:: Lymphomatoid papulosis (LyP) is defined as a chronic recurrent skin disease characterized by waxing and waning papules and nodules with histologic features of a CD30-positive T-cell lymphoma. Three histological subtypes (A, B, and C) were already recognized, and only more recently, a further variant simulating histologically an aggressive epidermotropic CD8-positive T-cell lymphoma was described, which was named LyP type D by the authors. We report the case of a 38-year-old woman presenting with a 1-year history of recurrent self-healing papules and nodules, predominantly affecting her upper and lower limbs but also the face, including the lower lip, with no associated systemic symptoms. A biopsy from 1 lesion revealed an infiltrate of atypical lymphoid cells extending throughout the dermis with massive epidermotropism displaying a pagetoid reticulosis-like pattern and a CD8CD30 cytotoxic T-cell phenotype. The clinicopathologic features conformed to the newly described type D variant of LyP. Diagnostic studies did not reveal any systemic involvement, and the patient remains otherwise well with no active treatment. In the present report, we discuss the need for clinicopathologic correlation to establish an accurate diagnosis and its importance for an adequate management of these patients.