母细胞性浆细胞样树突细胞肿瘤2例并文献复习

doi:10.3969/j.issn.1004-583X.2024.03.010

摘要/Abstract

摘要：

目的探讨母细胞性浆细胞样树突细胞肿瘤的临床病理特征、治疗及预后。方法结合相关文献,回顾性分析2例母细胞性浆细胞样树突细胞肿瘤患者的临床表现、组织学形态、免疫表型、治疗方案及预后。结果 2例组织形态均符合母细胞性浆细胞样树突细胞肿瘤的病理改变,免疫组织标记CD4、CD56、CD123、TCL1阳性,排除淋巴系、髓系及NK细胞来源,2例EBER原位杂交检测结果均为阴性;例l累及骨髓及淋巴结,对症支持治疗,2月后死亡;例2经CHOP方案化疗8周期,半年后病情进展,全身多发红疹,后予吉西他滨、奥沙利铂及培门冬酶化疗9周期,目前病情稳定。结论母细胞性浆细胞样树突细胞肿瘤多以皮肤症状为首发,具有独特的临床病理特点,诊断依靠组织学及免疫表型,目前尚无有效的治疗方案且预后差。

关键词: 母细胞性浆细胞样树突细胞肿瘤, 病理学, 临床, 免疫表型分型, 治疗

Abstract:

Objective To explore the clinical and pathological characteristics, treatment, and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN). Methods Based on literature review, we retrospectively analyzed clinical manifestations, histological morphology, immune phenotype, treatment plan, and prognosis of two patients with BPDCN. Results The tissue morphology of both cases was consistent with the pathological changes in BPDCN. Immunohistochemical markers cluster of differentiation (CD)4, CD56, CD123, T-cell leukemia-1 (TCL1) were positively stained, and lymphoid, myeloid, and NK cell sources were ruled out. Epstein-Barr virus-encoded RNA (EBER) in situ hybridization results of both cases were negative. Case 1 involved bone marrow and lymph nodes, received symptomatic supportive treatment, and died 2 months later. Case 2 was managed by 8 cycles of chemotherapy with the cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) regimen. The condition of Case 2 progressed at 6 months with multiple rubella throughout the body. Later, gemcitabine, oxaliplatin, and pemetrexed chemotherapy were administered for 9 cycles. Currently, the condition of Case 2 was stable. Conclusion BPDCN often have skin symptoms as the first symptom and unique clinical and pathological characteristics. Diagnosis of BPDCN relies on histology and immunophenotype. There is currently no effective treatment plan, leading to a poor prognosis of BPDCN.

Key words: blastic plasmacytoid dendritic cell neoplasm, pathology, clinical, immunophenotyping, treatment

中图分类号:

R733

任雷, 刘晔, 鲍书友, 李葵芳. 母细胞性浆细胞样树突细胞肿瘤2例并文献复习[J]. 临床荟萃, 2024, 39(3): 253-258.

Ren Lei, Liu Ye, Bao Shuyou, Li Kuifang. Blastic plasmacytoid dendritic cell neoplasm: Two cases and literature review[J]. Clinical Focus, 2024, 39(3): 253-258.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2024.03.010

https://huicui.hebmu.edu.cn/CN/Y2024/V39/I3/253

图/表 10

图1 额部皮损,淡紫红色结节,稍隆起,质稍硬

Fig.1 Skin lesion located on the forehead, with slightly raised and hard light purple-red nodule

图2 瘤组织位于真皮内 (HE,低倍)

Fig.2 Invasive growth of tumor cells in dermis (HE, low power field)

图3 肿瘤细胞浸润性生长 (HE, 中倍)

Fig.3 Infiltration of tumor cells into striated muscle tissue (HE, middle power field)

图4 无亲表皮现象(HE,中倍)

Fig.4 No epidermal affinity (HE, middle power field)

图5 高倍镜下细胞单一,小-中等大,核型极不规则,呈卵圆形,可见小核仁,核分裂相可见,胞质淡染(HE染色,高倍镜下)

Fig.5 High power microscopic showed that the cells are single, small to medium large, with extremely irregular and oval karyotype, small nucleolus, mitotic phase, and light staining of the cytoplasm (HE staining, High power microscopic)

图6 CD56阳性表达(免疫组化染色,Polymer Conjugate法)

Fig.6 CD56 positive expression (immunohistochemical staining, Polymer Conjugate method)

图7 CD4阳性表达 (免疫组化染色,Polymer Conjugate法)

Fig.7 CD4 positive expression (immunohistochemical staining, Polymer Conjugate method)

图8 CD123阳性表达(免疫组化染色,Polymer Conjugate法)

Fig.8 CD123 positive expression (immunohistochemical staining, Polymer Conjugate method)

图9 背部皮损 a.病变进展前; b.病变进展后, 可见多个浅灰色至淡红色斑块

Fig. 9 Skin lesions on the back a. Back skin before lesion progression; b. Multiple light gray to light red plaques can be seen on the skin of the back after lesion progression

图10 手臂皮损 a.病变进展前;b.病变进展后,可见多个淡红色斑块

Fig.10 Skin lesions on the arm a.Arm skin before lesion progression;b.Arm skin after lesion progression, multiple reddish patches are seen

参考文献 13

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