中华皮肤科杂志 ›› 2013, Vol. 46 ›› Issue (12): 851-854.

• 论著 • 上一篇    下一篇

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

徐教生1,刘元香1,徐哲2,张立新3,徐子刚4,高子芬5,马琳1   

  1. 1. 首都医科大学附属北京儿童医院
    2. 国际儿童医学中心 首都医科大学附属北京儿童医院皮肤科
    3. 首都医科大学附属北京儿童医院皮肤科
    4. 北京首都医科大学附属北京儿童医院皮肤科
    5. 北京大学基础医学院病理学系
  • 收稿日期:2013-01-15 修回日期:2013-07-22 发布日期:2013-12-01
  • 通讯作者: 张立新 E-mail:zhanglixinpifuke@163.com

Primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome in a child

Jiao-Sheng XU1,Yuan-xiang LIUZhe XU 3, 3,   

  • Received:2013-01-15 Revised:2013-07-22 Published:2013-12-01

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摘要： 【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

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

Abstract: XU Jiao-sheng*, LIU Yuan-xiang, XU Zhe, ZHANG Li-xin, XU Zi-gang, GAO Zi-fen, MA Lin. *Department of Dermatology, Beijing Children′s Hospital, Capital Medical University, Beijing 100045, China Corresponding author: ZHANG Li-xin, Email: zhanglixinpifuke@aliyun.com 【Abstract】 A 13-year-old girl presented with multiple recurrent cutaneous plaques for more than six months, which had been aggravated with intermittent fever for five months. No obvious systemic abnormality was found. Dermatological examination revealed multiple, non-ulcerative, painless, infiltrative, indurated, poorly marginated, purple subcutaneous plaques measuring 3 － 11 cm in diameter with slight squamation in bilateral buttocks and lower limbs. Laboratory investigations showed bicytopenia with the white blood cell count being （0.03 - 3.7） × 109/L and red blood cell count being （2.8 - 4.4） × 1012/L, a normal platelet count, hypofibrinogenemia （1.79 g/L） and low proportion of natural killer cells （4.6%）. Bone marrow smear showed active proliferation of cells, decreased proportion of granulocytes, presence of a few indefinitely classified cells, and phagocytosis. Reticulocytes were easily seen in the bone marrow smear. Pathologically, no obvious abnormality was observed in the epidermis or dermis, while the subcutaneous adipose tissue, especially fat lobules and some interlobular septa, was extensively infiltrated by large- to medium-sized lymphoid cells with pleomorphic and twisted nuclei as well as a small amount of cytoplasm; necrosis and phagocytosis of nuclear debris and lymphocytes were visible. The atypical lymphoid cells stained positive for CD3, T-cell intracellular antigen-1, granzyme B and TCRγδ with partial loss of CD5 and CD7, but negative for CD56, CD4, CD8 and TCRαβ. No Epstein-Barr virus-encoded RNA （EBER） was detected by in situ hybridization. Based on these findings, a diagnosis of primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome was made. 【Key words】 Lymphoma, T-cell, cutaneous; Lymphohistiocytosis, hemophagocytic

引用本文

徐教生 刘元香 徐哲 张立新 徐子刚 高子芬 马琳. 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[J]. 中华皮肤科杂志, 2013,46(12):851-854. doi:

Jiao-Sheng XU Yuan-xiang LIU Zhe XU. Primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome in a child [J]. Chinese Journal of Dermatology, 2013, 46(12): 851-854.doi: