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.     

结外鼻型NK/T细胞淋巴瘤

报告1例结外鼻型NK／F细胞淋巴瘤。患者男,43岁。左面部红肿1个月余。初发时表现为左下眼睑红肿,以后红肿范围渐扩大,累及整个左侧面部,并有水疱形成甚至破溃。根据皮损组织病理检查和免疫组化染色结果,确诊为结外鼻型NK／T细胞淋巴瘤。     

TCR gene-rearranged,extranodal NK/T-cell lymphoma,nasal type,presenting as gyrate patches

In the CD56+ cutaneous nasal-type NK/T-cell lymphoma strongly associated with latent EBV infection, subcutaneous or dermal nodules are the most common skin findings, but great morphologic heterogeneity has been noted including papules, infiltrated plaques, and ulcerated tumors, and TCR genes are mostly germline. We describe a case of nasal and nasal-type NK/T-cell lymphoma featuring multiple erythematous polycyclic patches on the trunk, which is similar to patch stage mycosis fungoides or other cutaneous T cell lymphoma. Immunohistochemical study of a skin biopsy specimen revealed CD2+, CD3epsilon+, CD56+, and CD45RO+ expression in the neoplastic cells. In situ hybridization using an anti-sense Epstein Barr virus early regions probe showed a positive reaction. However, clonal TCR beta gene rearrangement was found.     

Leukocytoclastic vasculitis with IgA deposits in angioimmunoblastic T cell lymphoma

Angioimmunoblastic T cell lymphoma (AILD) is a type of peripheral T cell lymphoma associated with fever and generalized lymphadenopathy. Cutaneous manifestations are seen in approximately 40% of the patients. We report herein a Japanese male patient with AILD associated with generalized purpura. The histology of the purpura included leukocytoclastic vasculitis with IgA deposits, which is rare in this disease. Using in situ hybridization and PCR methods, we showed that the involved lymph node was positive for Epstein-Barr virus and that the purpura was negative.     

伴嗜酸性粒细胞增多的结外NK/T细胞淋巴瘤一例

患者,男,53岁。反复双下肢结节红斑1年余,发热1个月余。入院前查血常规嗜酸性粒细胞 4.92×109/L(55.3%),首诊为“嗜酸性粒细胞增多症”。入院后查EB病毒DNA阳性,下肢皮损病理检查：以血管为中心较致密的单一核细胞浸润,有异型性改变,及数量较多的嗜酸性粒细胞浸润。免疫组化：CD3（+）,Ki67（80%）,TIA1（+）,Granzyme B（+）,Perforin（+）,EBER（+）。鼻黏膜及喉肿物活检见异型细胞。诊断：结外NK/T细胞淋巴瘤。     

Nasal natural killer/T cell lymphoma with cutaneous involvement: case report and Chinese literature review reported in China mainland

Nasal natural killer (NK)/T cell lymphoma is an Epstein-Barr virus (EBV) associated lymphoma that arises in the nasal area and aggressively invades surrounding tissues. Our patient was a 48-year-old male who had had nasal obstruction and nasal discharge for 2 years and infiltrating plaques and necrosis on his nasal dorsum for three months. He developed fever and fatigue two weeks before admission. Biopsy from both skin and nasal mucosa revealed atypical medium-sized tumor cells infiltrating into the dermis. Immunohistochemical studies revealed that the tumor cells were UCHL-1, cytoplasmic CD3, CD56, TIA-1, and granzyme B positive, and CD8 and CD20 negative. In situ hybridization for EBV-DNA was positive. Clonal TCRb and TCRg gene rearrangement were negative. The patient was treated with cyclophosphamide, vincristine, and prednisone (COP) and with local radiotherapy, but he died 20 days later. We reviewed the cases of nasal NK/T cell lymphoma reported in mainland China in the Chinese literature during the last 5 years.     

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( )。     

鼻部NK/T细胞淋巴瘤累及皮肤

为了探讨鼻部NK／T细胞淋巴瘤累及皮肤的临床表现、病理、免疫表型及EB病毒相关性。通过临床表现及病程,分析原发及累及部位。通过UCHL－1,CD56,多克隆CD3,CD8,CD20,TIA－1,粒酶B（GrB）等对肿瘤细胞免疫表型进行研究,EBV－EBER探针原位杂交检测肿瘤细胞阳性率分析其病因及发病。结果本例皮肤及鼻部取材组织病理表现为坏死及中等大小的异型性肿瘤细胞增生浸润,UCHL－1＋,CD56＋,CD3＋,CD8－,CD20－,TIA－1＋,granzyme B ,为NK／T细胞淋巴瘤表型。EBV瘤细胞阳性率达60％,进一步证实为鼻部NK／T细胞淋巴瘤,EBV相关性。支持鼻部NK／T细胞淋巴瘤是具有特殊免疫表型,与EB病毒有高度相关性的独立疾病,临床进展迅速,病程短,提示为高度侵袭性。     

Extranodal NK / T-cell lymphoma with cutaneous involvement: 'nasal' vs. 'nasal-type' subgroups— a retrospective study of 18 patients

Background  Extranodal natural killer T (NK/T) cell lymphoma is subcategorized into 'nasal' and 'nasal-type' NK/T-cell lymphomas according to the primary sites of anatomical involvement.
Objectives  The aim of this study was to characterize the cutaneous manifestations of the skin involving extranodal NK/T-cell lymphoma and to define the distinctive features of 'nasal' and 'nasal-type'. In addition, the prognostic factors that affect overall survival were investigated.
Methods  A retrospective case study of 18 patients with extranodal NK/T-cell lymphoma with cutaneous involvement was performed.
Results  The NK/T-cell lymphomas usually occurred in middle-aged, male patients. Most of the patients presented with either cellulitis or ulcer. A facial predilection for the location of the lesion was noted. The characteristic features of the 'nasal-type' compared with the 'nasal' were a localized involvement of the skin, less aggressive clinical course and better survival outcome.
Conclusions  Extranodal NK/T-cell lymphomas are extremely aggressive regardless of their subgroup. However, the 'nasal-type' NK/T-cell lymphoma was clinically less aggressive, more localized and had a better outcome compared with the other type. Cellulitis and ulcer were the major cutaneous manifestations.     

皮肤结外NK/T细胞淋巴瘤10例临床及组织病理特征分析

目的:分析皮肤结外NK/T细胞淋巴瘤临床及组织病理特点。方法:收集2012年10月——2019年5月该院皮肤科诊治的10例皮肤结外NK/T细胞淋巴瘤患者,对其皮损、皮损组织病理及免疫组化等进行回顾性分析,并随访患者生存情况。结果:所有患者中男5例及女5例。4例皮损位于四肢,3例位于鼻、面部,3例泛发全身,皮损均表现为红斑、结节、溃疡及水疱。本组患者皮损组织病理均表现为不典型的淋巴细胞浸润,不规则折叠核,肿瘤细胞围绕血管中心性生长,血管阻塞破坏及凝固性坏死,可浸润至真皮及皮下组织。原位杂交示浸润的不典型淋巴细胞EB病毒编码RNA(EBER)(10例)胞核阳性。免疫组化示T细胞胞质内抗原(TIA)-1(10例)、CD3(5例)和颗粒酶B(7例)胞质阳性,CD56(6例)、CD30(3例)、CD8(2例)和CD20(2例)胞膜阳性,增殖核抗原(Ki-67)(10例)胞核约5%～90%阳性。10例患者中6例死亡,发病至死亡时间5～75个月。结论:皮肤结外NK/T细胞淋巴瘤疾病进展快,皮损泛发者提示预后不良。     

Epstein-Barr virus-associated primary central nervous system lymphoma in a patient with adult T-cell leukemia / lymphoma

We present a case of Epstein-Barr virus (EBV)-associated primary central nervous system lymphoma (PCNSL) arising from a patient with cutaneous-type adult T-cell leukemia/lymphoma (ATLL). Extranodal sites affected by ATLL include the skin, lung, liver, gastrointestinal tract and central nervous system (CNS). CNS involvement usually occurs as an acute and lymphoma-type ATLL. PCNSL is a rare type of tumor and the vast majority of PCNSL are of B-cell lineage. Individuals with acquired, iatrogenic or congenital immunodeficiency are at increased risk of PCNSL, which is commonly associated with EBV. In our patient, the expression of latent infection membrane protein 1 (LMP1), EBV nuclear antigen 2 (EBNA2), and EBV-encoded small RNA (EBER) in tumor cells confirmed a type III latency of EBV infection. Human T-cell lymphotropic virus type I (HTLV-I) can induce immunodeficiency before the overt development of ATLL. The HTLV-I infection led to suppression of the immune system and the development of EBV-associated PCNSL. This is the first reported case of the clinicopathological features of EBV-associated PCNSL arising from a patient with ATLL.     

误诊为丹毒的结外NK/T细胞淋巴瘤一例

结外NK/T细胞淋巴瘤(ENKTL)是一种罕见的侵袭性非霍奇金淋巴瘤亚型。本文报道以面部肿胀为表现的结外NKT细胞淋巴瘤一例,患者曾多次被误诊为“丹毒、蜂窝组织炎”等,最终通过病理、免疫组化等检查确诊,给予DDGP方案（培门冬酶、吉西他滨、顺铂、地塞米松）化疗,随访9个月患者恢复良好。