發(fā)布時(shí)間：2018-04-14 14:34

  本文選題：皮損表現(xiàn) + 母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤��； 參考：《山西醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的:總結(jié)母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤(BPDCN)的診斷要點(diǎn)、治療和預(yù)后,提高臨床醫(yī)生對(duì)BPDCN的認(rèn)識(shí),為進(jìn)一步明確診斷、治療及判斷預(yù)后提供依據(jù)。方法:整理2012年1月至2016年12月山西省腫瘤醫(yī)院血液科診治的4例BPDCN患者的病例資料,包括一般信息、臨床特征、組織病理學(xué)表現(xiàn)、免疫組化結(jié)果、其他輔助檢查結(jié)果、治療方案及預(yù)后情況等,回顧性分析整理的資料,并結(jié)合有關(guān)文獻(xiàn)進(jìn)行討論。結(jié)果:4例BPDCN患者中3例為女性,1例為男性,年齡分別是66、15、63及59歲,平均發(fā)病年齡為50.8歲。4例患者均以皮損為首發(fā)癥狀,1例僅累及皮膚,余3例均累及皮膚、骨髓和淋巴結(jié),其中2例累及中樞神經(jīng)系統(tǒng)。3例行皮膚活檢,組織病理結(jié)果:均在真皮層可見中等大小的異型性細(xì)胞,未累及表皮層;1例行淋巴結(jié)活檢,組織病理結(jié)果:正常淋巴結(jié)結(jié)構(gòu)消失,可見到中等大小的淋巴樣細(xì)胞,無(wú)血管侵犯和壞死。4例患者的腫瘤細(xì)胞均表達(dá)CD123、CD4和CD56,4例患者的腫瘤細(xì)胞均不表達(dá)髓過(guò)氧化物酶(MPO)、CD20、CD3、CD30、CD79a、EB病毒編碼RNA(EBER)。4例BPDCN患者中1例放棄治療后失訪,3例接受化療,均初治效果較好,但很快復(fù)發(fā),其中2例因病情進(jìn)展累及中樞神經(jīng)系統(tǒng)(CNS)而死亡,其平均生存期為12.5個(gè)月,1例仍治療中。結(jié)論:BPDCN是一種罕見病,好發(fā)于老年男性,具有獨(dú)特的組織學(xué)、免疫表現(xiàn),常以皮損為首發(fā)表現(xiàn),容易漏診、誤診,且呈高度侵襲性,病情進(jìn)展較快,預(yù)后極差,其診斷需結(jié)合臨床特征、組織病理學(xué)以及免疫組織化學(xué)標(biāo)記作出綜合判斷。該病發(fā)病率低,目前尚沒(méi)有統(tǒng)一的治療標(biāo)準(zhǔn)。
[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.
【學(xué)位授予單位】：山西醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R733

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