母细胞性浆细胞样树突细胞肿瘤4例临床分析并相关文献复习

发布时间：2018-04-14 14:34

本文选题：皮损表现 + 母细胞性浆细胞样树突细胞肿瘤　；参考：《山西医科大学》2017年硕士论文

【摘要】：目的:总结母细胞性浆细胞样树突细胞肿瘤(BPDCN)的诊断要点、治疗和预后,提高临床医生对BPDCN的认识,为进一步明确诊断、治疗及判断预后提供依据。方法:整理2012年1月至2016年12月山西省肿瘤医院血液科诊治的4例BPDCN患者的病例资料,包括一般信息、临床特征、组织病理学表现、免疫组化结果、其他辅助检查结果、治疗方案及预后情况等,回顾性分析整理的资料,并结合有关文献进行讨论。结果:4例BPDCN患者中3例为女性,1例为男性,年龄分别是66、15、63及59岁,平均发病年龄为50.8岁。4例患者均以皮损为首发症状,1例仅累及皮肤,余3例均累及皮肤、骨髓和淋巴结,其中2例累及中枢神经系统。3例行皮肤活检,组织病理结果:均在真皮层可见中等大小的异型性细胞,未累及表皮层;1例行淋巴结活检,组织病理结果:正常淋巴结结构消失,可见到中等大小的淋巴样细胞,无血管侵犯和坏死。4例患者的肿瘤细胞均表达CD123、CD4和CD56,4例患者的肿瘤细胞均不表达髓过氧化物酶(MPO)、CD20、CD3、CD30、CD79a、EB病毒编码RNA(EBER)。4例BPDCN患者中1例放弃治疗后失访,3例接受化疗,均初治效果较好,但很快复发,其中2例因病情进展累及中枢神经系统(CNS)而死亡,其平均生存期为12.5个月,1例仍治疗中。结论:BPDCN是一种罕见病,好发于老年男性,具有独特的组织学、免疫表现,常以皮损为首发表现,容易漏诊、误诊,且呈高度侵袭性,病情进展较快,预后极差,其诊断需结合临床特征、组织病理学以及免疫组织化学标记作出综合判断。该病发病率低,目前尚没有统一的治疗标准。
[Abstract]:Objective: to summarize the main points of diagnosis, treatment and prognosis of blastoplasmacytoid dendritic cell tumor (BPDC), and to improve clinicians' understanding of BPDCN so as to provide evidence for further diagnosis, treatment and prognosis.Methods: from January 2012 to December 2016, the data of 4 cases of BPDCN, including general information, clinical features, histopathological manifestations, immunohistochemical results and other auxiliary examination results, were collected and analyzed.The treatment plan and prognosis were retrospectively analyzed and discussed.Results of the 4 cases of BPDCN, 3 cases were female and 1 case was male. The mean age of onset was 50.8 years old. The average age of onset was 50.8 years old. The first symptom was skin lesions in 1 case, and the skin, bone marrow and lymph nodes in the other 3 cases.Skin biopsy was performed in 2 cases involving the central nervous system (CNS). Histopathological results showed that moderate size of abnormal cells were found in the dermis, and lymph node biopsy was performed in 1 case without involvement of the epidermis. The histopathological results were as follows: normal lymph node structure disappeared.You can see medium-sized lymphoid cells,The tumor cells of 4 patients without vascular invasion and necrosis were all expressed CD123T CD4 and 4 patients with CD56 were not expressed myeloperoxidase (MPO), CD20, CD30, CD30, CD79ahe virus encoding RNA(EBER).4. In one case of BPDCN, 1 case received chemotherapy after giving up treatment, and 3 cases received chemotherapy, and the initial treatment effect was better.But it recurred quickly, 2 of them died as a result of CNS. The average survival time was 12.5 months and 1 case was still under treatment.Conclusion: BPDCN is a rare disease, which is prone to occur in old men. It has unique histologic and immunological features. It is often characterized by skin lesions as the first manifestation, misdiagnosis, misdiagnosis, high invasive, rapid progression and poor prognosis.The diagnosis should be combined with clinical features, histopathology and immunohistochemical markers.The incidence of the disease is low and there is no uniform standard of treatment.
【学位授予单位】：山西医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R733

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