中华皮肤科杂志 ›› 2024, e20230118.doi: 10.35541/cjd.20230118

• 研究报道 • 上一篇    下一篇

4例伴有DUSP22重排且具有双相组织学模式的原发皮肤CD30阳性淋巴增殖性疾病临床病理分析

田翠翠1    张莹2    孙建方2    甘璐3    李昱4    陈浩2    

  1. 1北京协和医学院  中国医学科学院  皮肤病医院，南京  210042；2中国医学科学院  北京协和医学院皮肤病医院病理科，南京  210042；3中国医学科学院  北京协和医学院皮肤病医院性病科，南京  210042；4重庆大学附属肿瘤医院病理科，重庆  400030
  • 收稿日期:2023-03-03 修回日期:2024-09-14 发布日期:2024-10-30
  • 通讯作者: 甘璐；李昱；陈浩 E-mail:467091016@qq.com; liyu100@cqu.edu.cn; ch76ch@163.com
  • 基金资助:
    南京市国家级临床医学中心培育计划项目（2019060001）；江苏省自然科学基金（BK20220214）

Clinicopathological analysis of four cases of primary cutaneous CD30-positive lymphoproliferative disorders with DUSP22 rearrangement exhibiting a biphasic histological pattern

Tian Cuicui1, Zhang Ying2, Sun Jianfang2, Gan Lu3, Li Yu4, Chen Hao2   

  1. 1Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing 210042, China; 2Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China; 3Department of Sexually Transmitted Disease, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China; 4Department of Pathology, Chongqing University Cancer Hospital, Chongqing 400030, China
  • Received:2023-03-03 Revised:2024-09-14 Published:2024-10-30
  • Contact: Gan Lu; Li Yu; Chen Hao E-mail:467091016@qq.com; liyu100@cqu.edu.cn; ch76ch@163.com
  • Supported by:
    Nanjing Incubation Program for National Clinical Research Center （2019060001）; Natural Science Foundation of Jiangsu Province （BK20220214）

PDF

6

PDF (Mobile)

0

摘要： 【摘要】 报道4例伴有双特异性磷酸酶蛋白22（DUSP22）重排且具有双相组织学模式的原发皮肤CD30阳性淋巴增殖性疾病患者并分析其临床病理学特征。4例患者于2018年至2022年至中国医学科学院皮肤病医院就诊或会诊，其中女性3例，男性1例，年龄51岁至73岁，皮损表现为丘疹或结节，其中2例为单发病变，2例为多发病变；累及部位分别为额头、下颌、双前臂、颈部及四肢。组织病理学呈双相浸润模式，即肿瘤同时出现亲表皮现象及真皮内浸润；1例可见Pautrier微脓肿形成，真皮内均无明显炎症细胞浸润；免疫组化显示，CD30呈双相模式，即在表皮内弱阳性表达，而真皮内强阳性表达；3例肿瘤细胞为CD3+CD4-CD8-表型，1例为CD3+CD4+CD8+表型；Ki67阳性率70% ~ 90%；所有病例均不表达B细胞标志、细胞毒蛋白和间变淋巴瘤激酶。荧光原位杂交检测显示所有患者均存在干扰素调节因子4（IRF4）-DUSP22分离信号。通过观察、局部切除、放疗或系统药物治疗，4例患者病变均消退。

关键词: CD30阳性淋巴增殖性疾病, DUSP22, 双相组织学模式, 临床病理分析

Abstract: 【Abstract】 To report four cases of primary cutaneous CD30+ lymphoproliferative disorders （pcCD30+LPDs） with DUSP22 rearrangement exhibiting a biphasic histological pattern. The clinicopathological features of pcCD30+LPD patients were analyzed. Four patients visited the Hospital of Dermatology of the Chinese Academy of Medical Sciences from 2018 to 2022 for treatment or consultation, including 3 female patients and 1 male patient, aged 51 to 73 years, with skin lesions manifesting as papules or nodules. Solitary lesions were found in 2 patients, and multiple lesions were observed in the other 2 patients. The affected sites were the forehead, lower jaw, forearms, neck and limbs. Histopathological examination revealed a biphasic pattern characterized by a diffuse infiltration of lymphocytes in both the dermis and epidermis. Pautrier-like microabscesses were observed in 1 case. There was no obvious inflammatory background in the dermis. An immunohistochemical study revealed a biphasic pattern of CD30 expression, with strong diffusion in dermal tumor cells, but weaker staining in the epidermal counterpart. CD3+CD4-CD8- phenotype T cells were found in 3 patients, and the remaining patient had a CD3+CD4+CD8+ phenotype. All cases had a high proliferative rate （70% - 90%） by Ki-67 staining. The cytotoxic marker, B cell markers and anaplastic lymphoma kinase were uniformly absent. Fluorescence in situ hybridization assay for the DUSP22-IRF4 locus on 6p25.3 showed a split signal in all patients. By observation, local resection, radiotherapy, or systemic drug therapy, the lesions all subsided in the 4 patients.

Key words: Lymphoma, T-cell, cutaneous, Lymphoma, primary cutaneous anaplastic large cell, Lymphomatoid papulosis, CD30 positive lymphoproliferative disorders

引用本文

田翠翠 甘璐 张莹 孙建方 李昱 陈浩. 4例伴有DUSP22重排且具有双相组织学模式的原发皮肤CD30阳性淋巴增殖性疾病临床病理分析[J]. 中华皮肤科杂志, 2024,e20230118. doi:10.35541/cjd.20230118

Tian Cuicui, Zhang Ying, Sun Jianfang, Gan Lu, Li Yu, Chen Hao. Clinicopathological analysis of four cases of primary cutaneous CD30-positive lymphoproliferative disorders with DUSP22 rearrangement exhibiting a biphasic histological pattern[J]. Chinese Journal of Dermatology,2024,e20230118. doi:10.35541/cjd.20230118