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1例

患者,男,64岁,躯干部丘疹1个月,皮疹逐渐增多,瘙痒明显,淋巴结肿大伴高热。淋巴结病理检查可见病理性核分裂象。免疫组化示：Ki-67标记指数约90%、CD21及CD23（示FDC网结构紊乱）、CD3及CD5（+）、CD34（血管+）、CD30（免疫母细胞+）、BCL-2（+）。 诊断：血管免疫母细胞性T细胞淋巴瘤。     

一例非霍奇金血管免疫母细胞性T细胞淋巴瘤并发心包积液患者的护理

<正>血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T-cell lymphoma,AITL)是一种罕见的外周T细胞淋巴瘤,约占其16.8%,占非霍奇金淋巴瘤的1%~2%~([1])。此病进展快,预后差,极易误诊且多见于亚洲人群~([2])。血管免疫母细胞性T细胞淋巴瘤临床表现很复杂,多为高热、浅表淋巴结肿大、皮疹、肝脾肿大等多种症状~([2,3])。由于非霍奇金血管免疫母细胞性     

第三讲原发性皮肤大细胞淋巴瘤的分类

原发性皮肤大细胞淋巴瘤（PCLC）可分为T细胞性和B细胞性两类。T细胞表型PCLCL[PCLCL(T)]可以CD30阳性与否预期其预后。CD30^＋PCLCL(T)和CD30^ 非皮肤原发性LCL（T）的预后差。从蕈样肉芽肿转化成的CD30^ 皮肤LCL的预后一般差，从淋巴瘤样丘疹病演变成的CD30^ -LCL仅系统性的预后差而皮肤CD30^ -LCL的预后则不差。原发性皮肤多形T细胞性淋巴瘤，大细胞型和原发性皮肤T－免疫母细胞性淋巴瘤的预后差。B细胞表型原发性皮肤大细胞淋巴瘤中绝大多数为原发性皮肤滤泡中心细胞性淋巴瘤，其预后较淋巴结滤泡中心细胞性淋巴瘤为好。     

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...     

以环形红斑为初发表现的T淋巴母细胞性淋巴瘤的临床研究

目的 的降雨 例罕见的以环形红斑为初发表现的成人T淋巴母细胞性淋巴瘤。方法 对其临床,组织病理,免疫组化,分子生物学和各种特殊的实验室检查进行研究。结果 颈部,腹股沟肿大数10个淋巴结,全身百余个环形红斑,胸部CT示;前上纵隔占位性淋巴瘤表现。胸水中找到异形细胞。骨髓片示;淋巴瘤累及骨髓,外周血中未发现瘤细胞。淋巴结结构破坏,瘤细胞弥漫分布,呈“满天星”图像。部分瘤细胞浸润,部分向表皮性,可见Pautrier微脓肿,少数淋巴样细胞细胞核呈曲核型,与淋巴结的瘤细胞相似;免疫组化示：LCA,CD43,UCHL-1均阳性,TdT阴性,原位杂交示EBER1/2在淋巴结组织中呈最性;皮肤组织中呈阴性。结论 此例为首例报告罕见的以环形红斑为初发表现的T淋巴母细胞性淋巴瘤。     

C型淋巴瘤样丘疹病1例并文献复习

患者男,18岁。躯干及四肢泛发丘疹并结节1月余。患者前胸、后背及四肢散在分布直径0.5~2.0cm大的丘疹及结节,部分皮疹顶端可见坏死及结痂。淋巴结B超提示双侧颈部及左侧腋窝多发淋巴结可见。左下肢皮损组织病理示真皮全层血管及附属器周围大量单一核细胞呈"楔形"浸润,部分细胞核大并可见核丝分裂相;免疫组化示CD30、CD4、CD8、CD68、Bcl-2、Bcl-6、TIA-1阳性(+),CD3、CD20、CD23、CD56、ALK-、PAX5及EBER原位杂交阴性(-),Ki-67(50%阳性)。符合C型淋巴瘤样丘疹病。每日予口服强的松片20mg及每周口服甲氨蝶呤10mg,1个月后皮疹明显减少和面积明显缩小。淋巴瘤样丘疹病属于原发性皮肤CD30+淋巴增生性疾病,有自限性,目前研究认为淋巴瘤样丘疹病组织学可分为A,B,C,D和E五型。该病一般预后较好,通常无需治疗。     

弥漫性大B细胞淋巴瘤皮肤受累一例

患者,女,76岁,全身皮下多发结节伴右下肢皮肤溃烂2个月。皮损病理活检示（腹壁肿物）：非霍奇金淋巴结,B细胞型,免疫表型符合DLBCL,生发中心来源。免疫组化：CD20弥漫连片（+）,CD3散在（+）,CD10（+）,CD21显示FDC网消失,Ki67（80%）。胸腹部增强CT示胃窦十二指肠占位性病变,胃镜检查取胃窦部病变活检示：（胃窦小弯、大弯）非霍奇金B细胞淋巴瘤,考虑DLBCL或其他高侵袭性类型;免疫组化：粘膜腺体间弥漫的淋巴样细胞侵润性生长,CD20连片阳性,Ki67（90%）。我院血液科诊断为非霍奇金淋巴瘤（弥漫大B细胞型）IV期IPI,评分5分 继发皮肤受累。患者家属拒绝治疗,患者出院1周后死亡。     

Sézary综合征3例

Ｓéｚａｒｙ综合征３例黄岚①陈明华①冯树芳①Ｓéｚａｒｙ综合征（Ｓéｚａｒｙｓｙｎｄｒｏｍｅ，ＳＳ），或称皮肤Ｔ细胞淋巴瘤综合征、Ｔ细胞淋巴瘤性红皮病，系皮肤Ｔ细胞淋巴瘤的一种类型。临床上以红皮病伴剧痒、掌跖角化、浅表淋巴结和肝、脾肿大，以及血中白细...     

成人T细胞白血病／淋巴瘤与皮肤淋巴瘤的鉴别

伴皮肤损害的成人Ｔ细胞白血病／淋巴瘤（ＡＴＬＬ）与皮肤Ｔ细胞淋巴瘤有极相似的临床病理和免疫分型，为了区别两者，对４例伴皮肤损害的ＡＴＬＬ和１８例皮肤淋巴瘤进行临床病理、免疫学分型及嗜人Ｔ淋巴细胞病毒Ｉ型（ＨＴＬＶ－Ｉ）前病毒ＤＮＡ的比较性研究，２例光线性类网织细胞增多症和２例皮肤淋巴细胞浸润症为阴性对照。结果：４例ＡＴＬＬ出现皮肤损害的同时表现系统症状如：广泛的浅淋巴结肿大，乳酸脱氢酶和白介素２受     

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 composite lymphoma of mycosis fungoides and Hodgkin lymphoma: Response to sequential therapy

Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphoma clones that occur in the same tissue site. The occurrence of cutaneous composite lymphoma (CCL) is extremely rare. Here we report a unique case of CCL consisting of Hodgkin lymphoma (HL) and mycosis fungoides (MF). Our patient presented with longstanding erythematous plaques on the skin and later developed axillary lymph node enlargement. Histopathologically, the skin lesions were characterized by a dense dermal lymphocytic infiltrate with prominent epidermotropism of pleomorphic T-cells, consistent with typical MF. Nonetheless, scattered large atypical cells resembling Reed-Sternberg (R-S) cells were interspersed among these atypical T-cells in the deep dermis. Immunophenotyping suggested a HL origin of these R-S cells. Monoclonality of T-cell receptor beta gene was detected in the skin, monoclonal immunoglobulin heavy chain gene rearrangement was identified in these R-S cells microdissected from the deep dermis, confirming the origin from HL. The lymph node biopsy showed nodular sclerosis classic Hodgkin lymphoma. Therefore, CCL of HL and MF, with lymph node HL was diagnosed. The lesions of this patient responded to a sequential treatment to HL and MF. Being aware of this rare CCL facilitates correct diagnosis and proper clinical management.     

Peripheral T-cell lymphoma involving subcutaneous tissue

The peripheral T-cell lymphomas, presumably derived from various immunocompetent peripheral T-cell system components, form a heterogeneous group of non-Hodgkin's lymphomas. We describe two patients with peripheral T-cell lymphoma primarily involving subcutaneous tissue. They presented with multiple subcutaneous nodules. Skin biopsy specimens in both patients demonstrated a lobular subcutaneous infiltrate. The infiltrate consisted of small and medium-sized atypical lymphoid cells. Both patients had a protracted clinical course before they were diagnosed as having malignant lymphoma. We detected latent Epstein-Barr virus infection in the skin lesions of case 2. Latent Epstein-Barr virus infection might be related to the development of this variant of peripheral T-cell lymphoma.     

CONGENITAL ICHTHYOSIS: CONCURRENT IMMUNODEEICIENCY AND ATYPICAL T CELLS

A patient with congenital ichthyosis and progressive neurologic anomalies showed disturbances in the specific humoral and cellular defense as well as the presence of atypical lymphoid cells in skin and lymph node. The latter resembled the atypical T cells found in mycosis fungoides and Sézary syndrome. The possibility of the presence of either a cutaneous T cell lymphoma or unregulated T cell stimulation leading to concurrent immunodeficiency in this patient is discussed.     

Cutaneous angiocentric T-cell lymphoma associated with Epstein-Barr virus.

BACKGROUND: Two unusual cases of cutaneous angiocentric T-cell lymphoma were found to be associated with Epstein-Barr virus infection. OBJECTIVE: The objective was to study the clinical course and the response of the disease to conventional chemotherapy. METHODS: Histologic specimens from both patients were studied. Clonal proliferation was assessed by Southern blot hybridization. RESULTS: The disease in both patients was rapidly progressive and responded poorly to aggressive treatment. Biopsy specimens showed infiltration of atypical lymphoid cells with angiocentricity and angiodestruction, which probably resulted in the observed tissue necrosis. Clonal proliferation of Epstein-Barr virus DNA was detected in tissue from primary skin lesions and disseminated nasal lesions. CONCLUSION: Epstein-Barr virus-associated angiocentric T-cell lymphoma in our patients was characterized by an aggressive course and resistance to conventional chemotherapy. A search for Epstein-Barr virus and the human T-lymphotropic virus should be performed in patients with atypical features of cutaneous T-cell lymphoma.     

Cutaneous involvement in the lymphoepithelioid variant of peripheral T-cell lymphoma, unspecified (Lennert lymphoma). Report of a case and review of the literature

Lennert lymphoma (LL), or the lymphoepithelioid variant of peripheral T-cell lymphoma, is an uncommon entity with rarely seen or reported presentations in the skin. Cutaneous involvement of LL has been characterized by asymptomatic, non-ulcerated, red to violet papules, nodules and small plaques (less than 5 cm) on the trunk and extremities. Histologically, there are localized cellular lymphoid infiltrates in the dermis that tend to localize around blood vessels or skin appendages. Key to the diagnosis of LL is the presence of epithelioid histiocytes and atypical small lymphoid cells without increased vascularity or epidermotropism. Immunophenotyping shows a dense monoclonal T-cell population commonly associated with aberrant loss of T-cell-associated antigens. T-cell receptor gene rearrangements are also identified. Patients typically present with advanced stage and have a low 5-year survival. Herein, we present a case of cutaneous involvement by LL at the time of initial presentation that persisted after initiation of chemotherapy and was finally verified as secondary cutaneous involvement of LL 1 year later histologically, immunophenotypically and by T-cell receptor gene rearrangement studies.     

Mycosis fungoides bullosa

A case of mycosis fungoides bullosa is presented. The results in our study confirmed that the predominant atypical lymphoid cells in the bullae, peripheral blood, and involved lymph nodes expressed the T-cell helper phenotype using immunophenotyping techniques. The literature is reviewed, confirming that our case demonstrated cells of the T-helper phenotype, not only in the skin but also in the blood and lymph node tissue. Bullous lesions in mycosis fungoides are rare.     

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.     

中国人皮肤型成人T细胞白血病/淋巴瘤一例报道

目的 报道1例皮肤型成人T细胞白血病/淋巴瘤(cATLL)。方法 检测皮肤损害的组织病理、直接免疫荧光和免疫表型的特点,同时应用酶联免疫吸附试验、蛋白印迹法以及聚合酶链反应分别检测患者外周血中嗜人T淋巴细胞病毒Ⅰ型(HTLV-Ⅰ)抗体和淋巴样细胞的HTLV-Ⅰ前病毒DNA。结果 此例患者的皮肤损害多形,除表现丘疹、斑块外,并发生疱壁紧张或松弛的大疱。组织病理检查示表皮下水疱,疱底真皮乳头和疱边缘处小至中等大不典型淋巴样细胞浸润。不典型淋巴样细胞示CD45⁺、CD45RO⁺,直接免疫荧光检查阴性。外周血中HTLV-Ⅰ抗体阳性;淋巴样细胞中HTLV-Ⅰ前病毒DNA阳性。患者最后死亡,病程1年10个月。结论 ATLL在我国并非极罕见,cATLL也存在,应加以警惕。     

A rare case of the cutaneous form of adult T-cell leukaemia/lymphoma: assessment of remission by PCR for clonal T-cell receptor gamma gene rearrangements in an electron beam-irradiated cutaneous lesion

Adult T-cell leukaemia/lymphoma is a lymphoproliferative disorder aetiologically associated with human T-cell lymphotropic virus type I infection. A cutaneous lesion often develops in the disease, and in rare cases, is even the only manifestation. Here we report a rare case of 'cutaneous' adult T-cell leukaemia/lymphoma with neither atypical cells in the peripheral blood nor lymph node involvement. All nodular lesions were completely eliminated after local electron beam irradiation (20 Gy/nodule in total). To evaluate whether or not there were residual lymphoma cells in the skin, we performed PCR to detect clonal T cell receptor gamma gene rearrangements. The sample from the nodule before irradiation showed evidence of a rearranged band, which was not detected at the same site after treatment nor in any peripheral blood. The findings suggest that this procedure is useful for the evaluation of therapeutic effects and the early detection of lymphoma recurrence.