儿童原发性皮肤γδ-T细胞淋巴瘤合并噬血细胞综合征一例

【摘要】 患儿女,13岁。臀部、四肢反复起斑块6个月,加重伴间断发热5个月。体检：各系统检查未见明显异常。皮肤科情况：臀部及双下肢散在分布直径3 ～ 11 cm质硬、浸润性、紫红色皮下斑块,界不清,表面少许脱屑,无明显压痛及溃疡形成。实验室检查：白细胞（0.03 ～ 3.7） × 109/L,红细胞（2.8 ～ 4.4） × 1012/L,血小板计数正常;纤维蛋白原1.79 g/L,NK细胞比例4.6%;骨髓涂片示骨髓增生活跃,粒系比例降低,网织细胞易见,并见少量分类不明细胞及噬血现象。皮损组织病理学检查：表皮、真皮未见明显异常,皮下脂肪组织内可见大量淋巴样细胞浸润,以脂肪小叶为主,部分小叶间隔受累;浸润细胞体积中等偏大,胞质少,核多形,部分核扭曲;可见坏死及吞噬核碎片、淋巴细胞现象;免疫组化：CD3、颗粒酶B、T细胞细胞内抗原-1（TIA-1）、TCRγδ阳性,CD5、CD7部分阳性,CD4、CD8、TCRαβ、CD56阴性,Epstein-Barr病毒编码RNA原位杂交检测阴性。诊断：原发性皮肤γδ-T细胞淋巴瘤合并噬血细胞综合征。 【关键词】 淋巴瘤,T细胞,皮肤; 淋巴组织细胞增多症,嗜血细胞性     

T小细胞型原发性皮肤间变性大细胞淋巴瘤1例

患者女 ,2 3岁 ,以孤立皮肤结节并溃疡为首发表现 ,溃疡面大而深 ,局部淋巴结肿大、溃烂坏死 ,反复新发 ,难以愈合 ,且病情进展迅速 ,伴多系统侵袭 ,皮肤病理及免疫组化示淋巴瘤 ,T细胞来源 ,CD3 0 (Ki 1)阳性 ,分期属IV期。予放化疗联合治疗 ,无效死亡 ,总病程 9个月。本文重点讨论本病的诊断、病理免疫学分类及相关预后等。     

复发/难治性皮肤T细胞淋巴瘤的非化疗药物新选择

随着对皮肤T细胞淋巴瘤研究的不断深入, 传统靶向药物、免疫检查点抑制剂、组蛋白去乙酰化酶抑制剂以及异基因造血干细胞移植等各种治疗手段不断出现, 均在临床试验中显示出良好的疗效及安全性。本文综述近年复发/难治性皮肤T细胞淋巴瘤非化疗药物的相关研究进展。     

蕈样肉芽肿治疗中国专家共识(2023)

蕈样肉芽肿(MF)是皮肤T细胞淋巴瘤最常见类型, 早期仅累及皮肤, 进展期出现肿块并有淋巴结、外周血和内脏受累。治疗前需对患者进行分期评估, 根据分期选择治疗方案。我国12位MF诊疗领域专家在国外最新指南和共识的基础上, 回顾了治疗方法的循证医学级别, 结合我国现状, 在MF一线、二线治疗方案和新疗法等方面达成共识, 为规范MF治疗提供指导。     

皮肤恶性T细胞性淋巴瘤伴反应性噬血组织细胞增生症的临床病理研究

报道6例皮肤T细胞性恶性淋巴瘤伴噬血组织细胞增生症.病变局限于真皮和皮下脂肪组织,灶性的肿瘤性T淋巴细胞浸润和分化良好的噬血组织细胞增生伴各种炎症细胞浸润.4例表现为病情进行性恶化、复发或死亡.并探讨其发病机理、诊断标准及鉴别诊断.     

原发性皮肤CD30+间变性大细胞淋巴瘤1例

患者女,84岁.右上肢肿块,逐渐增大,破溃;后右侧腋窝出现皮下肿块,右侧背部出现钱币状紫红色肿块6个月,患处无明显自觉症状.系统检查:一般情况可,无肿大淋巴结,各系统检查无明显异常.组织病理及免疫组织化学结合临床诊断为原发性皮肤间变性大细胞淋巴瘤.     

细胞治疗遗传性大疱性表皮松解症研究进展

遗传性大疱性表皮松解症(EB)是一种罕见的残毁型、致死型单基因遗传性皮肤病, 对社会和家庭造成了沉重负担。细胞疗法因其卓越稳定的临床疗效成为EB治疗中极有前景的一种手段, 本文从干细胞和体细胞两方面总结近年来细胞疗法在EB的实验室研究及临床应用方面的进展。     

原发性皮肤CD30^＋间变性大细胞淋巴瘤1例

患者女,84岁。右上肢肿块,逐渐增大,破溃;后右侧腋窝出现皮下肿块,右侧背部出现钱币状紫红色肿块6个月,患处无明显自觉症状。系统检查：一般情况可,无肿大淋巴结,各系统检查无明显异常。组织病理及免疫组织化学结合临床诊断为原发性皮肤间变性大细胞淋巴瘤。     

西达本胺联合苦参碱对皮肤T细胞淋巴瘤细胞系增殖、凋亡的影响及可能的凋亡机制

目的研究西达本胺联合苦参碱对皮肤T细胞淋巴瘤(CTCL)细胞系的增殖抑制和凋亡诱导作用, 探讨其凋亡机制。方法采用0.4 μmol/L西达本胺、0.6 g/L苦参碱单药或联合分别作用HH、Hut78细胞24、48、72 h后, MTS法检测HH、Hut78细胞增殖率。二甲基亚砜(DMSO)处理HH、Hut78细胞作为对照组。西达本胺、苦参碱单药或联合作用两细胞系48 h后, 流式细胞仪检测HH和Hut78细胞凋亡率, Western免疫印迹法检测各组细胞凋亡相关蛋白的表达。统计分析采用重复测量、单因素方差分析, 组间两两比较采用LSD-t检验。结果与对照组相比, 西达本胺、苦参碱单药或联合均能一定程度抑制HH、Hut78细胞增殖(F = 15.88、558.26, P < 0.05、< 0.001)。48 h时, Hut78细胞系苦参碱组(20.98% ± 1.53%)、西达本胺组(22.44% ± 7.74%)和联合组细胞凋亡率(44.53% ± 1.85%)均显著高于对照组(8.42% ± 4.23%;LSD-t = 4.76、5.31、13.69, 均P < 0...     

Spitz样肿瘤研究进展

Spitz痣、非典型Spitz肿瘤和Spitz样黑素瘤的临床及组织学特征有重叠, 三者鉴别极具挑战。该文结合Spitz样肿瘤的临床和组织学特征, 综述其免疫组化特点和荧光原位杂交检测方法在本组疾病中的研究进展。     

原发皮肤CD30阳性间变大细胞淋巴瘤七例临床病理分析

目的 探讨原发皮肤CD30阳性间变大细胞淋巴瘤（PC-ALCL）的临床与组织病理学特征。 方法 回顾性分析7例PC-ALCL患者的临床及病理资料情况。 结果 7例患者中,男6例,女1例,平均发病年龄52岁。皮损为红色结节、肿块和（或）斑块,3例多发,4例单发,6例伴溃疡形成。所有患者均未见系统受累。组织病理改变：肿瘤细胞在真皮内弥漫性分布,细胞体积大,胞质丰富,胞核呈异形改变,可见核分裂象。肿瘤细胞CD30和细胞毒蛋白阳性,CD20、CD56、间变性淋巴瘤激酶（ALK）和EB病毒编码小RNA原位杂交均阴性。结论 PC-ALCL是一种少见的原发于皮肤的低度恶性T细胞淋巴瘤,结合临床表现、皮损组织病理及免疫组化检查可确诊,罕见系统受累及转移。     

伴噬血细胞综合征的皮肤NK/T细胞淋巴瘤鼻型一例

患者男,48岁,反复面颈、躯干、四肢丘疱疹10年,加重10 d,发热4 d。皮损组织病理检查示真皮内大量小到中等异形淋巴样细胞浸润,以血管及附属器周围明显,伴血管结构的破坏,并可累及皮下脂肪小叶间隔。免疫组化标记示真皮及皮下组织浸润的异形细胞CD45RO（+++）、CD3（-）、CD4（-）、CD8（+）、CD56（+）、TIA-1（++）、粒酶B（+）、EBER（++）、CD3ε胞质（++）、CD20（-）、CD79a（-）、CD30（-）。实验室检查示外周血红细胞、白细胞、血小板进行性下降,转氨酶、胆红素持续性升高,高甘油三酯血症及纤维蛋白原降低,腹部B超示肝脾肿大。诊断：皮肤NK/T细胞淋巴瘤鼻型伴噬血细胞综合征。     

CD30-positive cutaneous T-cell lymphoma with concurrent solid tumour

Extranodal CD30+ T-cell lymphomas seldom carry classical t(2;5) translocation and are usually anaplastic large cell lymphoma kinase protein negative. They cover a wide spectrum of histological and clinical behaviour. The prognosis of CD30+ cutaneous T-cell lymphoma (CTCL) is good in the absence of nodal primary or disseminated disease. These lesions can undergo spontaneous regression, and overlap with the group of lesions of lymphomatoid papulosis. Although an increased incidence of solid tumours has been reported in patients with CD30+ non-Hodgkin lymphoma of the skin, reports of concurrent malignancies are rare in CD30+ CTCL. We report two patients with CD30+ CTCL who, respectively, had concurrent disseminated gastric carcinoma and bilateral ovarian teratoma. Despite an aggressive clinical and histological appearance, both cases ran favourable clinical courses. The CTCL responded completely to chemotherapy in one patient, who eventually succumbed to gastric cancer. In the other patient, lesions regressed spontaneously after bilateral oophorectomy. A possible relationship between the lymphoma and the solid tumours is discussed.     

原发性皮肤T淋巴细胞瘤的治疗选择

原发性皮肤T细胞淋巴瘤是一组原发于皮肤的淋巴细胞异常增殖性疾病,蕈样肉芽肿是其中最常见的类型.原发性皮肤T细胞淋巴瘤的治疗选择,主要取决于世界卫生组织-欧洲癌症研究和治疗组织-国际皮肤淋巴瘤学会制定的TNMB分期与分类标准,其他需要考虑的因素包括,治疗方法的有效率、起效与持续缓解时间,治疗耐受性与相关毒副作用,治疗实施的难易程度及治疗费用等.原发性皮肤T细胞淋巴瘤早期主张支持治疗和局部治疗为主,晚期则要进行联合化疗,或根据病情辅以免疫调节治疗.一些特异的细胞因子、单克隆抗体和融合毒素也逐步试用于治疗原发性皮肤T细胞淋巴瘤.     

原发性皮肤间变性大细胞淋巴瘤

报告1例原发性皮肤间变性大细胞淋巴瘤.患者女,24岁.左手中指红斑33 d,根据皮损组织病理及免疫组化标记结果诊断为原发性皮肤间变性大细胞淋巴瘤.     

原发性皮肤CD30+间变性大细胞淋巴瘤1例

报告1例原发性皮肤CD30+间变性大细胞淋巴瘤.患者男,72岁.右足拇趾红肿,外侧有一2.0 cm×2.5 cm红色溃疡面,伴血性液体渗出,近端有一1.5 cm×2.0 cm暗红色结节,表面结痂.皮损组织病理及免疫组化标记确诊为原发性皮肤CD30+间变性大细胞淋巴瘤.     

Gemcitabine treatment in cutaneous T-cell lymphoma: a multicentre study of 23 cases

Background  Primary cutaneous T-cell lymphomas (CTCLs) are malignancies characterized by a clonal T-cell infiltrate involving the skin. CTCLs often show resistance to conventional antineoplastic chemotherapy. Gemcitabine is a pyrimidine analogue which has shown efficacy and a favourable safety profile in solid tumours and haematological malignancies.
Objectives  We report a multicentre retrospective study of 23 patients who received gemcitabine for advanced-stage CTCL and emphasize the high incidence of serious unusual adverse events.
Methods  We collected data from 23 patients with refractory CTCL (14 mycosis fungoides, six Sézary syndrome and three other CTCL). Gemcitabine was given weekly within a 21- or 28-day schedule. Response was evaluated after three and six cycles of chemotherapy. For each patient, all adverse events were recorded.
Results  Of the 16 patients who received at least three cycles of gemcitabine, 10 achieved a response (62·5%). Only five patients reached the sixth cycle of treatment and four still had a favourable response. Haematological toxicity was recorded in 15 cases with severe grade 3 or 4 neutropenia in seven patients (30%) and six serious infections (26%). Other serious adverse events were observed in six cases (26%): one haemolytic–uraemic syndrome, one severe capillary leak syndrome, one acute heart failure related to cardiac arrhythmia, two bullous and erosive dermatitis, and one recurrent influenza-like syndrome with altered general condition.
Conclusions  Our study confirms the early efficacy of gemcitabine in advanced-stage CTCL. However, our results contradict the safety profile of gemcitabine previously reported and underline the high incidence of severe complications including visceral and cutaneous involvement.     

Ofuji papuloerythroderma evolving to cutaneous T-cell lymphoma

Ofuji papuloerythroderma is an uncommon entity of unknown aetiology, characterized by a pruritic eruption of widespread, red–brown, flat papules that leads to spare skin folds. A number of cases have been described associated with tumour pathology, mainly cutaneous T‐cell lymphomas. We report a new case of Ofuji papuloerythroderma evolving to cutaneous T‐cell lymphoma in an 85‐year‐old woman who had been previously diagnosed with papuloerythroderma 7 years previously.     

Primary cutaneous CD30-positive large cell lymphoma

Primary cutaneous CD30-positive large cell lymphoma is a recently described entity, recognized by immunohistochemical criteria. Spontaneous regression may occur and although relapses are frequent, most patients have a favourable prognosis. We describe a 83-year-old woman with a large ulcerated tumour on her right forearm, with 2 months evolution. Histological and immunohistochemical examination revealed a CD30-positive large cell lymphoma. Systemic involvement was not detected. The tumour regressed spontaneously. Cutaneous relapses occurred one year later, all with spontaneous regression. After a 2-year follow-up period, the patient is well, without concurrent extracutaneous disease. This case is in keeping with other reports of spontaneous regression and favourable outcome of this type of cutaneous lymphoma.     

皮肤T细胞淋巴瘤表观遗传学研究进展

表观遗传学主要是研究在不改变DNA序列的情况下,发生的基因表达水平可遗传的变化,主要包括DNA甲基化、组蛋白修饰和非编码RNA调控.皮肤T细胞淋巴瘤是一组原发于皮肤的T淋巴细胞恶性增殖性疾病,蕈样肉芽肿和Sezary综合征是最常见的皮肤T细胞淋巴瘤.近年来研究表明,表观遗传学不仅在皮肤T细胞淋巴瘤的发生发展中起重要作用,而且可以通过改变表观遗传达到治疗皮肤T细胞淋巴瘤目的.