原发性皮肤γδT细胞淋巴瘤并发噬血细胞性淋巴组织细胞增多症1例  

A case of primary cutaneousγδT cell lymphoma complicated with hemophagocytic lymphohistiocytosis

作　　者：汤竣弛 黄雪沂 张文雍[3] 何琳[3] 胡海涛 杨斌[1] TANG Junchi;HUANG Xueyi;ZHANG Wenyong;HE Lin;HU Haitao;YANG Bin(Guangdong Clinical College of Dermatology,Anhui Medical University,Guangzhou 510091,China;Shunde Hospital,Guangzhou Medical University,Foshan 528315,China;Nanfang Hospital,Southern Medical University,Guangzhou 510515,China)

机构地区：[1]安徽医科大学广东皮肤病临床学院,广东广州510091 [2]广州医科大学附属顺德医院,广东佛山528315 [3]南方医科大学南方医院,广东广州510515

出　　处：《皮肤性病诊疗学杂志》2023年第1期65-70,共6页Journal of Diagnosis and Therapy on Dermato-venereology

摘　　要：报告原发性皮肤γδT细胞淋巴瘤并发噬血细胞性淋巴组织细胞增多症1例。患者女,33岁,左上臂结节1个月,下肢红斑伴发热1周。皮肤科检查:左上臂内侧皮肤红肿伴硬结节形成,约6~8 cm,皮温升高,触痛明显,中央可见一窦道渗出黄色透明液体。双下肢近踝部散在十余个大小不等红色结节性斑块,边界欠清,有明显压痛。皮损组织病理示真皮层异型淋巴样细胞浸润,免疫组化示CD3(+)、CD4(-)、CD5(+)、CD8(+)、CD56(-)、颗粒酶B(+)、TIA-1(+)、TCRγδ(+),Ki-67(约90%+)。骨髓穿刺活检示粒系成熟障碍,红系增生明显活跃,网状细胞中可见噬血细胞。TCRG基因重排检测在目标条带范围内可见单克隆性扩增峰。PET-CT示全身多发淋巴结增大。诊断:原发性皮肤γδT细胞淋巴瘤并发噬血性淋巴组织细胞增多症。予CHOP+VP-16联合化疗方案2周后,体温恢复正常,皮损逐渐消退,遂定期随访。随访时患者症状多次反复,复查仍反复有新发病灶,多次更改化疗方案未见明显好转,遂于1年后行造血干细胞移植术。现患者症状控制平稳,仍在随访中。We report a case of primary cutaneousγδT cell lymphoma complicated with hemophagocytic lymphohistiocytosis.A 33-year-old female presented with nodule on her left arm for 1 month and erythema on her both legs for a week.Physical examination revealed redness and swelling of the left upper arm with nodule sized 6.8 cm,accompanied with elevated skin temperature and tenderness.A sinus with yellow exudate was observed in the center of the lesion.There were more than 10 red nodular plaques in various sizes with unclear boundaries and tenderness around the ankles.Histology showed atypical lymphoid infiltrate in the dermis.Immunohistochemistry showed positive for CD3,CD5,CD8,Granzyme B,TIA-1,TCRγδand Ki67(90%).Bone marrow aspiration showed abnormal maturation of granulocytes,erythroid hyperplasia and hemophagocytosis by reticular cells.TCRG gene rearrangement showed clonal amplification peak in the target band.PET-CT revealed multiple lymphadenopathy in her body.The diagnosis was primary cutaneous Gamma-Delta T-Cell lymphoma with hemophagocytic lymphohistiocytosis.After 2 weeks of CHOP+VP-16 combined with chemotherapy,patient′s body temperature became normal,and the skin lesions gradually subsided.During the regular follow-up,the patient′s symptoms recurred many times,and new skin lesions repeatedly developed,without significant improvement after the treatments with different chemotherapy.One year later,patient received hematopoietic stem cell transplantation,and her condition became stable.Currently the patient is still under follow-up.

关 键 词：皮肤T细胞淋巴瘤 ΓΔT细胞 噬血综合征 

分 类 号：R739.5[医药卫生—肿瘤]