皮肌炎伴血管免疫母细胞性T细胞淋巴瘤一例

患者，女，82岁，因面、颈、胸背部红斑2月，加重伴双上肢肌痛无力1月于我科就诊，根据患者临床表现及辅助检查诊断为皮肌炎，因患者合并全身多发淋巴结肿大，结合组织病理、免疫组化和基因重排检测诊断为皮肌炎伴发血管免疫母细胞性T细胞淋巴瘤。予以甲强龙静滴及化疗，随访2月后去世。     

T免疫母细胞性淋巴瘤1例

临床资料患者,男,76岁,农民。主因躯干、四肢皮肤多发红色肿物半年、右侧阴囊肿物3个月就诊。患者于半年前无意中发现躯干、四肢皮肤多个肿物,大小不一,呈红色,半球形,高出皮肤,无疼痛瘙痒等不适,     

以风团为表现的血管免疫母细胞性T细胞淋巴瘤1例

患者男,39岁,反复全身风团伴发热,浅表、后腹膜及盆腔多发肿大淋巴结4个月,淋巴结活检、免疫组织化学及T细胞淋巴瘤克隆基因重排确诊血管免疫母细胞性T细胞淋巴瘤.以风团样皮疹为首发表现的血管免疫母细胞性T细胞淋巴瘤少见,对于常规疗效不佳且伴有多发异常肿大淋巴结病例,结合全面体检及相关实验室病理检查,可早期发现,以免误诊漏...     

以播散性带状疱疹为首发表现的血管免疫母细胞性T细胞淋巴瘤1例

患者男,68岁,右侧头部疱疹伴疼痛1周,躯干、四肢皮疹3 d。既往身体健康。体格检查:右侧头部可见成簇疱疹,部分破溃,部分已结痂。双侧颈部及双侧腋窝可触及肿大淋巴结。躯干及四肢散在红色丘疹,上有小水疱、周围绕以红晕,部分结痂。皮损组织病理示:淋巴结活检可见淋巴结结构破坏,未见明显淋巴滤泡,淋巴结内多形性细胞浸润,瘤细胞生长活跃,核分裂象易见。淋巴结免疫组织化学显示CD3广泛阳性;CD10片状阳性;BCL6片状阳性;CXCL13广泛阳性;Ki-67(70%~80%阳性);PD1广泛阳性;EBER散在阳性。     

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。     

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。     

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.     

伴过敏样皮疹的血管免疫母细胞性T细胞淋巴瘤一例

患者男,67岁,以周身皮疹1月余、发热1周为主诉入院.1个月前双大腿出现红斑样皮疹,伴胸闷、气短、呼吸困难,于外院诊为荨麻疹、过敏性肺炎给予抗生素、甲泼尼龙40 mg静脉滴注,2 d后皮疹消退,症状减轻,甲泼尼龙渐减量.治疗期间发现颈部、腋下、腹股沟多枚肿大淋巴结,行淋巴结穿刺后诊为淋巴结结核.1周前开始给予抗结核治疗,患者出现发热,最高39.8℃,皮疹复现并泛发周身.为进一步诊治来我院.体检:颜面潮红,躯干、四肢泛发轻度水肿性红斑,大多融合成片,颈部、腋下、腹股沟触及多枚甲大至杏核大淋巴结,部分淋巴结融合,质硬,移动度差.     

皮肤血管内T细胞淋巴瘤:1例报道及文献复习

对1例血管内淋巴瘤进行免疫表型分析及原位杂交检测与 EB病毒的关系,并复习相关文献患者男,32岁,发热2个月,左大腿斑块1月.全身体检及辅助检查血小板90× 109,血沉24mm/h,C反应蛋白28.9 mg/L,LDH 448 U/L.病理学检查肿瘤细胞位于真皮层的小血管内,瘤细胞体积较大、核仁明显、胞浆嗜碱性,核分裂相可见.瘤细胞免疫表型CD3,CD45( LCA)呈阳性表达.原位杂交显示E2BERs阴性.患者经CHOP联合化疗一个疗程后获得缓解.血管内T细胞淋巴瘤是一种罕见淋巴瘤,此瘤的诊断必须依赖病理学及免疫组织化学检查,在不明原因发热伴发皮肤斑块时,应及时活检,积极联合化疗.     

Angioimmunoblastic lymphadenopathy-type peripheral T-cell lymphoma with cutaneous infiltration: report of a case and its gene expression profile

Angioimmunoblastic T-cell lymphoma is a type of peripheral T-cell lymphoma that is clinically characterized by high fever and generalized lymphadenopathy with or without cutaneous involvement. A 55-year-old Japanese man presented with red papular lesions on the trunk and limbs, oedema, and generalized lymphadenopathy. Histological findings in the lymph nodes showed destructive germinal centres, proliferation of arborizing postcapillary venules, and atypical medium-sized lymphocytes. The cutaneous lesions also contained atypical lymphocytes. Immunohistochemical studies indicated that the neoplastic cells were mature CD4+ T lymphocytes. Southern blot analysis detected a clonal expansion of T-cell receptor beta. Based on these findings, a diagnosis of angioimmunoblastic T-cell lymphoma with cutaneous infiltration was made. Despite systemic chemotherapy, the disease exhibited a high level of activity and continued on a fatal course. An analysis of gene expression profiling using complementary DNA microarrays revealed significant expression of some chemokines and cytokines, e.g. secondary lymphoid tissue chemokine, macrophage inflammatory protein (MIP)-1beta, MIP-3alpha, MIP-3beta, B-lymphocyte chemokine, interleukin-16 and tumour necrosis factor-beta, and an apoptosis-inhibitory protein (FLICE inhibitory protein) in the affected lymph nodes. Profiling of gene expression patterns for a variety of genes in additional cases may be helpful in determining which factors predict the biological and clinical behaviour of angioimmunoblastic T-cell lymphoma or other aggressive malignant lymphomas.     

Systemic varicella zoster virus reinfection in a case of angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma that causes immunological disorders such as immunosuppression, autoimmune disease-like symptoms and allergy. We report a case of a 67-year-old man with AITL who had a serious varicella zoster virus (VZV) reinfection that appeared clinically to be varicella. Forty percent of cases of AITL are associated with skin rash. A variety of cutaneous manifestations have been reported; however, the majority are macropapular eruptions that are often diagnosed as drug associated. Our study emphasizes the need to correctly diagnose opportunistic infections, such as the varicella that is documented in our patient, at early stages in AITL.     

皮下脂膜炎样T细胞淋巴瘤1例

报告1例皮下脂膜炎样T细胞淋巴瘤。患者女,34岁。四肢出现疼痛性红斑、结节9年,伴有发热、消瘦等。组织病理学改变主要在皮下脂肪组织内,可见瘤细胞浸润于脂肪细胞间。免疫组化显示瘤细胞表达CD45Ro及CD68,不表达CD20。     

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.     

足部皮肤T细胞淋巴瘤1例

报告1例足部皮肤T细胞淋巴瘤。患者男,63岁。左足跟部肿胀4个月,抗感染治疗无效,皮肤组织病理和免疫组化均符合T细胞淋巴瘤。     

皮下脂膜炎样T细胞淋巴瘤

报告1例皮下脂膜炎样T细胞淋巴瘤。患者男,26岁。曾无防护地接触放射性元素钴3年。躯干部出现暗红色肿块1．5个月,伴有全血细胞减少。皮损组织病理学改变主要为真皮中下部及皮下脂肪层弥漫性瘤细胞浸润。免疫组化染色显示LCA（＋）、CD45RO（＋）、CD3（＋）、CD2（＋）、CD8（＋）、TIA-1（＋）、CD20（-）。     

皮下脂膜炎样T细胞淋巴瘤1例

报道1例皮下脂膜炎样T细胞淋巴瘤。患者女，34岁。全身反复出现肿块、结节性损害1年。4个月前部分肿块破溃，伴发热、乏力、贫血等症状。皮损组织病理显示皮下脂肪层的淋巴细胞、组织细胞浸润，脂膜炎样T细胞瘤。免疫组化证明下细胞来源，伴有TCRγ基因克隆性重排，诊断为脂膜炎样T细胞瘤。     

Subcutaneous T-cell lymphoma: report of two cases

We have seen two patients, a 24-year-old man and a 15-year-old girl, with subcutaneous nodules and fever. The histo-pathological findings in both cases showed a lobular, histiocytic panniculitis with 'bean bag' cells and lymphoid cells identified as immature T-cells. This picture was consistent with subcutaneous T-cell lymphoma associated with cytophagic histiocytic panniculitis. The girl died after 5 months of disease with florid hemophagocytic syndrome; the man was subjected to a course of treatment (CHOP; cyclophosphamide/doxorubicin/vincristine/prednisone) and after 6 months observation his symptoms of nodules and fever disappeared.     

儿童皮下脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征一例附文献复习

患儿,女,8岁。皮肤结节、斑块8个月,右大腿肿胀1个月,伴发热1周。肝脏肋下约1~2 cm可触及、脾脏肋下约2~3 cm可触及,质软,无触痛。实验室检查:血细胞降低,肝酶、血脂、乳酸脱氢酶、C反应蛋白升高,血沉加快,凝血功能异常,铁蛋白升高。免疫组化:CD3、CD8、颗粒酶B、βF1阳性,Ki-67:约50%(+)。骨髓活检:骨髓增生活跃。皮损病理检查:皮下脂肪见异型细胞。诊断为脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征。我们对相关文献进行了复习。