血管免疫母细胞性T细胞淋巴瘤伴发环形红斑1例

报告1例血管免疫母细胞性T细胞淋巴瘤伴发环形红斑皮疹病例.患者全身皮肤有大量环形红斑,红斑内缘有鳞屑.淋巴结组织结构大部分受破坏,有大小不等的瘤细胞浸润,血管增生及内皮细胞肿胀,并有多种细胞浸润,免疫表型CD10、bcl-6、CD21、Ki-67、CD45RO和CD3ε均阳性,可见TCRG基因克隆性重排,未见IgH基因克隆重排,EB病毒原位杂交:EBERs(偶+).骨髓检查:骨髓增生大致正常.皮损组织病理:表皮内见个别淋巴样的细胞浸润,表皮内可见多个水疱,疱内可见异型淋巴样细胞,核呈逗点状或多角形,见到Pautrier微脓肿;血管周围可见多量淋巴细胞及组织细胞浸润.免疫组化:CD4、CD8和 CD45RO均阳性,CD20阴性.根据患者的临床特点和实验室检查结果认为,此例血管免疫母细胞性T细胞淋巴瘤患者伴发的环形红斑可能属于一种非特异性皮炎反应.     

大疱性蕈样肉芽肿

蕈样肉芽肿(MF)是最常见的皮肤T细胞恶性淋巴瘤,但罕见大疱性损害.患者女,75岁,因皮肤瘙痒性红斑及水疱半年,于1986年5月就诊.入院时水疱泛发于躯干及手足,疱壁紧张,疱液清亮,基底有或无红斑,伴弥漫性秃发及脱眉,呈轻微狮面.皮损进一步发展,在手足出现鳞屑性红斑、肿瘤及广泛糜烂,左锁骨上淋巴结肿大.左下肢水疱活检显示表皮下水疱,疱内含有红细胞、纤维蛋白及异型淋巴细胞.此外,表皮内也见异型淋巴细胞的聚集.免疫组化染色显示Leu-4阳性,Leu-14阴性,证实为T淋巴细胞.左锁骨上淋巴结内的异型淋巴样细胞,其组织学和免疫组化染色均与皮肤所见相同.骨髓穿刺活检显示粒细胞系增     

以皮损为首发表现的淋巴母细胞淋巴瘤/白血病六例临床病理分析

目的 探讨以皮损为首发表现的淋巴母细胞淋巴瘤/白血病的临床、组织病理表现及免疫组化特点.方法 分析中国医学科学院皮肤病医院2012-2015年诊断的6例以皮损为首发表现的淋巴母细胞淋巴瘤/白血病的临床和组织病理特点.结果 6例患者中男4例、女2例;儿童和青年4例,成人2例.中位发病年龄13.5岁,平均病程8.5个月.皮损表现为单发(1例)或多发(5例)结节或浸润性斑块,组织病理学表现为真皮及皮下脂肪内形态单一、中等大小、胞质较少、染色质细腻的淋巴样细胞增生,可见小核仁,无亲表皮现象,1例可见星空现象.2例免疫学表型符合B淋巴母细胞淋巴瘤,2例符合T淋巴母细胞淋巴瘤,2例呈现T、B双系表型.该病治疗困难,1例患儿在化疗后获缓解,2例患者在接受化疗后皮损部分改善.结论 皮损表现和组织学无法区别B和T淋巴母细胞淋巴瘤,只有通过免疫表型进行鉴别,早期骨髓及影像学检查尤为重要.     

免疫母细胞性淋巴结病样T细胞淋巴瘤伴皮肤损害1例

免疫母细胞性淋巴结病样Ｔ细胞淋巴瘤（ＩＢＬ－ＴＣ）是特殊类型的周围Ｔ细胞淋巴瘤。临床特征为发热;淋巴结、肝脾肿大;皮疹及高γ球蛋白血症。皮疹呈多种形态,随其发展主要为二型：丘疹结节型和红皮病型。淋巴结病理示淋巴结结构破坏,有异形淋巴样细胞、免疫母细胞和浆细胞样的所谓淡染细胞（ｐａｌｅｃｅｌｌｓ）浸润。免疫学示Ｔ细胞标记。     

原发性皮肤间变性大细胞淋巴瘤1例

报告1例原发性皮肤间变性大细胞淋巴瘤。患者女,49岁。右小腿结节1年,溃烂5个月。皮损组织病理检查:真皮内有密集的淋巴样细胞浸润,瘤细胞大、核呈肾形或不规则形、核分裂像多见,免疫组化示瘤细胞约70?30阳性、约20?45Ro阳性,而CD3、CD20、MPO、TIA-1、ALK-1均为阴性。诊断为原发性皮肤间变性大细胞淋巴瘤。     

非霍奇金淋巴瘤1例

报告1例以皮肤肿瘤为首发表现的儿童非霍奇金淋巴瘤。患儿女,8岁。右侧鼻翼出现肿块3个月余,伴进行性增大1个月就诊。体格检查示局部淋巴结不增大,系统检查无异常。皮损组织病理检查示真皮内有异形淋巴样细胞浸润,免疫组化染色结果示：CD45RO（＋）,CD20,HMB45,CK,CD30和CD68均阴性,证实为T细胞淋巴瘤。     

间变性淋巴瘤激酶阴性的间变性大细胞淋巴瘤泛发性皮肤侵犯一例

患者男,37岁,入院前7个月无明显诱因右大腿出现一鹅蛋大小肿物,无明显不适,肿物逐渐增大,右大腿、臀部出现弥漫性、非凹陷性肿胀,入院前2个月全身皮肤出现暗红色丘疹、结节、斑块,部分斑块渐出现大小不一的糜烂、溃疡。实验室检查：白蛋白降低,乳酸脱氢酶显著升高。B超示浅表淋巴结肿大、融合,彩色多普勒示淋巴结内部较丰富的树枝样血流信号。CT显示右大腿及会阴部广泛淋巴结肿大伴软组织水肿,上腹部广泛淋巴结肿大,纵膈内淋巴结肿大。皮损组织病理：真皮全层致密分布单一核细胞,部分有异形性及不典型核分裂;免疫组化：CD3、CD8、CD30（阳性细胞占80%）、CD4、CD45RＯ、粒酶B 阳性,CD56、间变性淋巴瘤激酶（ＡＬＫ）、T细胞胞质内抗原1阴性。淋巴结病理：淋巴结结构完全破坏,肿瘤弥漫成片生长,肿瘤细胞比一般的大细胞淋巴瘤瘤细胞大,胞质丰富,嗜碱性或嗜双色性,细胞核偏位,呈马蹄形、肾形或分叶状,核染色质稀疏,可见单个或多个嗜碱性小核仁;免疫组化：CD2、CD4、CD3、粒酶B、上皮膜抗原（EMA）、Ki-67、CD30阳性,CD8、CD56、T细胞胞质内抗原（TIA）-1、ＡＬＫ均为阴性。诊断：间变性淋巴瘤激酶阴性的原发系统型间变性大细胞淋巴瘤泛发性皮肤侵犯。     

免疫母细胞性淋巴结病样T细胞淋巴瘤伴皮肤损害例

免疫母细胞性淋巴结产产Ｔ细胞淋巴瘤（ＩＢＬ－ＴＣ）是特殊类型的周围Ｔ细胞淋巴瘤。临床特征为发热；淋巴结、肝脾肿大；皮疹及高γ球蛋白症。皮疹呈多种形态，随其发展主要为二型；丘疹结节型和红皮病型。淋巴结是示淋巴结结构破坏，有形淋巴样细胞、免疫母细胞和浆细胞样的所谓淡染细胞（ｐａｌｅ ｃｅｌｌｓ）浸润。免疫学示Ｔ细胞标记。     

表现为播散性帕哲样网织细胞增生症的原发性皮肤γ/δT细胞淋巴瘤

作者首次报告一例原发性γ、δ~+皮肤T细胞淋巴瘤(CTCL)。患者,男性,65岁。皮肤上多个红斑鳞屑性斑片、斑块、结节及疼痛性溃疡性肿物1年。实验室检查:血相、骨髓相正常。周围血中T细胞亚群(CD_4和CD_8)和B细胞比例正常,T细胞受体(TCR)-δ-1~+淋巴细胞占周围血中单一核细胞的4%。皮损光镜检查显示表皮内有小至中等大小的非典型淋巴样细胞浸润,胞浆丰富、透明,核位于中央,呈圆或椭圆形,核染色质深。真皮浅层血管周围亦可见少许非典型淋巴样细胞。免疫组化示表皮内     

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.     

以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状的间变性大细胞淋巴瘤1例

患儿女,7岁,以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状,半年后左下肢出现皮下肿物确诊为间变性大细胞淋巴瘤。患儿首发临床表现为全身皮肤水疱、溃疡、结痂伴发热、腹股沟淋巴结肿大。皮损组织病理:表皮可见角化不良细胞,界面改变,表皮少量淋巴细胞浸润,未见异型细胞;淋巴结病理:淋巴结大片坏死,其间血管壁坏死,周围绕以多数淋巴样细胞;肿物病理:大量淋巴样细胞弥漫分布。免疫组织化学:CD30(+),ALK胞浆(+),CD5(-),CD7(+),CD4(+),CD8(-),TIA-1(+),CD2(+),LCA(+),EMA(+),CD3(-),Vimentin(+),Ki-67(>80%+)。诊断:间变性大细胞淋巴瘤。     

Lymphomatoid papulosis followed by large-cell lymphoma: immunophenotypical and genotypical analysis

The immunophenotype and genotype of atypical cells in skin and lymph node infiltrates were investigated in a patient with lymphomatoid papulosis (LyP) complicated by anaplastic large-cell lymphoma of the lymph nodes. The large atypical cells in both skin and lymph nodes displayed an almost identical immunophenotype, i.e. CD30+ and CD25+. Southern blot analysis for T-cell receptor beta-chain gene rearrangement revealed an identical gene configuration in DNA extracted from skin and lymph node. Our results strongly support the hypothesis that clonal populations of T cells arising in cutaneous LyP lesions may undergo malignant transformation, spread into regional lymph nodes, and give rise to secondary malignant lymphomas, such as anaplastic large-cell lymphoma.     

Cutaneous involvement in lymphoblastic lymphoma

Lymphoblastic leukemia/lymphoma (LBL) is a malignant neoplasm of precursor lymphocytes of B- or T-cell phenotype. Involvement of the skin is relatively uncommon. We examined retrospectively the clinicopathologic, immunophenotypic, and molecular genetic features of six patients with cutaneous involvement of LBL (B-LBL=5; T-LBL=1). Patients presented clinically with solitary, large tumors located on the head (3 cases) or the back (1 case), or with generalized tumors (2 cases). Ulceration was uncommon. In two patients the onset of skin lesions was concomitant to the diagnosis of lymphoblastic leukemia. Histopathologic examination showed in all cases a dense, diffuse, monomorphous infiltrate located in the entire dennis and subcutaneous fat. A typical "starry sky" pattern was observed in the majority of the lesions. In some areas neoplastic cells were aligned in a "mosaic-like" fashion. Cytomorphologically, medium sized lymphoid cells with round or convoluted nuclei, inconspicuous nucleoli and scant cytoplasm predominated. There were no significant differences in the histopathologic features of skin lesions in T- and B-LBL. In B-LBL, CD79a was more useful than CD20 in determining the phenotype of neoplastic cells (4/5 cases positive for CD79a as compared to 2/5 cases positive for CD20). TdT, CD10 and CD43 were positive in 4 cases, CD34 in 2. The case of T-LBL revealed positivity for CD1a, CD3, CD43 and TdT, and negativity for CD34 and for B-cell markers. All neoplasms were positive for CD99 and bcl-2, and showed a high proliferation rate. Molecular genetic analysis of J(H) and T-cell receptor (TCR) genes performed using a polymerase chain reaction technique revealed a monoclonal rearrangement of J(H) genes in all five B-LBLs. One of these cases showed also a concomitant TCR-gamma gene rearrangement. A monoclonal rearrangement of the TCR-gamma gene was detected in the case of T-LBL. Our study shows that skin lesions of LBL present characteristic clinicopathologic and molecular features allowing the differentiation from other cutaneous lymphomas, even in cases without clinical history of previous precursor lymphoblastic leukemia/lymphoma.     

An Autopsy Case of Angioimmunoblastic T-cell Lymphoma with a High Content of Epithelioid Cells in the Lymph Node: Immunohistochemical and Genomic Analyses

A 79-year-old female developed red papulonodular eruptions on her extremities, facial erythema, generalized lymphadenopathy and high fever. Histopathology of an affected lymph node showed the features of angioimmunoblastic T-cell lymphoma with a high content of epithelioid cells. She died about two years after the onset despite therapy. Genomic Southern blotting and immunostaining of the lymph nodes were performed twice. In August of 1993, Southern blotting did not show any rearrangement of the immunoglobulin or the T-cell receptor (TCR) gene. Small or medium-sized lymphoid cells were positive for CD4 or CD8 (CD4:CD8=2:1). However, in September of 1994 (at autopsy), rearrangements of TCR Cβ1, Jβ2 and Jγ genes were observed. Small or medium-sized lymphoid cells were positive for CD4, but negative for CD8. Several large cells were positive for Latent Membrane Protein 1 (LMP1) of the Epstein-Barr virus (EBV). Our results proved that selective oligoclonal proliferation of tumor cells (probably CD4+) accompanied the disease progress.     

鼻NK/T细胞淋巴瘤1例

报道1例以皮肤为首发症状的鼻NK／T细胞淋巴瘤。患者男，48岁。双下肢、躯干、双上肢陆续出现红色斑丘疹、结节及溃疡4月余，鼻腔发现新生物1月余。皮肤及鼻部组织病理示中、小淋巴样细胞弥漫浸润；免疫组化染色：CD56（＋），LCA（＋），UCLH－1（＋），26(-),CD57(-);EBER( )。     

Paraneoplastic Erythema Annulare Centrifugum Eruption: PEACE

Erythema annulare centrifugum is a reactive erythema of various possible etiologies including, although less often, an associated cancer. Cancer-related erythema annulare centrifugum is most commonly associated with lymphoproliferative malignancies, specifically lymphoma and leukemia. Malignancy-associated erythema annulare centrifugum is more frequently seen in women than men and the presence of skin lesions usually precedes the clinical diagnosis of the underlying malignancy. Neoplasm-derived erythema annulare centrifugum lesions often resolve following treatment of the cancer. Recurrence of erythema annulare centrifugum may occur along with the relapse of the underlying malignancy. Paraneoplastic erythema annulare centrifugum eruption (PEACE) is speculated to be a result of a cytokine or other tumor-associated factors.     

套细胞淋巴瘤伴皮肤虫咬样反应一例

【摘要】 患者男,74岁,确诊套细胞淋巴瘤5年,躯干、四肢丘疱疹伴瘙痒10个月就诊。皮疹瘙痒剧烈,给予抗组胺药物对症治疗不能缓解。体检：躯干、四肢皮肤见散在绿豆至黄豆大小红色丘疹及丘疱疹,部分表面见浅表结痂,以双上肢皮损为主,颈部、双侧腹股沟可触及肿大淋巴结,约2 cm × 1 cm。颈部淋巴结病理示,正常淋巴结结构完全破坏,中等大淋巴样细胞呈结节状或弥漫增生浸润,免疫组化示CD20（+++）,CD79α（+++）, Bcl-2（++）,细胞周期蛋白D1（+++）,CD5（++弱）,CD43（++）,Bcl-6（++）, PAX-5（+++）,κ（+++）,λ（±） ,Ki-67（10% ~ 30%+不均）。皮损组织病理及免疫组化：表皮大致正常,真皮浅中层血管、附属器周围见以中等大小淋巴样细胞为主的团灶状浸润,其间散在嗜酸性粒细胞;免疫组化：CD3（部分+）,CD5（弥漫性+）,CD20（部分+）,细胞周期蛋白D1（部分+）,Ki-67（10% +）。根据临床资料、淋巴结及皮损组织病理及免疫组化,诊断为套细胞淋巴瘤伴皮肤虫咬样反应。     

Activated-cytotoxic TCRαβ+CD4+ peripheral T-cell lymphoma with hypodermal localization: Case report of a lymphoproliferative disorder probably evolved from the CD4+ cytotoxic T-cell subpopulation

The World Health Organization (WHO) classification of hematopoietic and lymphoid tumors identifies distinctive subtypes of peripheral T-cell lymphoma (PTCL), and, additionally, some PTCLs involving mostly extranodal sites like the skin. The difficulty of classifying PTCLs according to the normal stages of T-cell differentiation and the lack of definitive diagnostic markers for most of the subtypes make the diagnosis of these diseases challenging. PTCL cases which do not fit into any of the specifically defined entities are categorized as PTCL not otherwise specified (PTCL-NOS). PTCLs-NOS represent less than 2% of the total cases of T-cell lymphoma involving the skin. This article illustrates a case of a PTCL-NOS in which tumor cells have an activated cytotoxic TCRαβ+CD3+CD4+CD56+ T-cell phenotype and histopathologic features of subcutaneous panniculitis-like T-cell lymphoma, leading to a fatal outcome.     

CD30 antigen expression in cutaneous inflammatory infiltrates of scabies: a dynamic immunophenotypic pattern that should be distinguished from lymphomatoid papulosis

BACKGROUND: Expression of CD30 antigen is a distinct marker of lymphocyte activation that was originally described in the Reed-Sternberg cells of Hodgkin's disease. The observation of CD30+ cells has been considered a diagnostic feature of cutaneous CD30 lymphoid proliferations. However, CD30 expression has also been reported in some cutaneous benign inflammatory infiltrates. METHODS: Eleven skin biopsies from patients with scabies were double-blindly and retrospectively analysed. A panel of histopathological parameters and immunophenotypic expression of CD4, CD8, CD30 and S-100 antigens was studied. CD30 and S-100 antigens expression were related to clinical features. RESULTS: Large CD30+ cells were demonstrated in eight (8/11) biopsies, corresponding to patients with long-standing lesions (3 months or longer). However, no expression of the CD30 antigen was observed in all biopsy specimens (3/11) corresponding to early lesions (2 months or less). The presence of S-100 positive cells in the papillary dermis was an almost constant feature. CONCLUSIONS: CD30+ large cells seem to be a common feature in long-standing infiltrates of scabies. CD30 expression in scattered cells of a cutaneous lymphoid infiltrate cannot be assessed as a strong diagnostic argument of neoplastic cutaneous CD30+ lymphoid proliferation (lymphomatoid papulosis/cutaneous CD30+ lymphoma). Therefore, the possibility that large atypical CD30+ cells may be also present in several benign inflammatory diseases should be always considered.