本文關(guān)鍵詞：母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤2例臨床病理特征分析及文獻回顧　出處：《吉林大學(xué)》2014年碩士論文　論文類型：學(xué)位論文

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【摘要】：母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤（blastic plasmacytoid dendriticcell neoplasm，BPDCN）是一種較為罕見的淋巴造血系統(tǒng)惡性腫瘤，常以皮膚損害為主要臨床表現(xiàn)，并可見淋巴結(jié)、軟組織、外周血、骨髓、甚至中樞神經(jīng)系統(tǒng)等侵犯。BPDCN具有較高的侵襲性，進展快，其診斷主要依據(jù)組織病理學(xué)及免疫病理學(xué)結(jié)果，又因其組織病理學(xué)改變?nèi)鄙偬禺愋�，必須結(jié)合全面的免疫病理學(xué)檢查，排除其他相似的造血系統(tǒng)惡性腫瘤方可診斷。所以診斷較難，易誤診。目前，尚無BPDCN的標(biāo)準(zhǔn)化治療方案，多采用急性白血病的化療方案。在疾病的初期，化療可以達到完全緩解（completeremission，CR），但多數(shù)患者很快復(fù)發(fā)。有研究表明，造血干細(xì)胞移植（stem cell transplantation, SCT）療效較好，其治療價值仍在探究中。該病預(yù)后較差。 目的：分析母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤的臨床表現(xiàn)、組織病理學(xué)特點及免疫病理學(xué)特點，為該病正確診斷及鑒別診斷提供進一步臨床依據(jù)。 方法：分析2例明確診斷為母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤患者的臨床特征，組織病理學(xué)及免疫病理學(xué)結(jié)果，回顧相關(guān)文獻，分析并總結(jié)。 結(jié)果：臨床表現(xiàn)：常見皮膚受累，多為青紫色丘疹、結(jié)節(jié)，可孤立或多發(fā)，偶有瘙癢、疼痛，還可見淋巴結(jié)、外周血及骨髓浸潤。病理學(xué)表現(xiàn)：表皮未見浸潤，真皮及皮下脂肪組織可見彌漫的母細(xì)胞樣腫瘤細(xì)胞浸潤，該細(xì)胞中等大小，形態(tài)較一致，胞質(zhì)稀少，細(xì)胞核大，卵圓形或不規(guī)則，表皮與真皮之間可見明顯的無細(xì)胞浸潤帶（Grens帶），未見血管浸潤及壞死。免疫病理學(xué)表現(xiàn)：CD4、CD56、CD123、CD31、CD43常表達，CD3、CD20、CD34、CD117、MPO、EBER均陰性。治療及預(yù)后：尚無標(biāo)準(zhǔn)化治療方案，該病進展較快，病情進行性惡化，疾病初期，化療有效，但易復(fù)發(fā)，預(yù)后差。 結(jié)論：母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤好發(fā)于中老年人。皮疹多為青紫色丘疹、結(jié)節(jié)，偶有瘙癢、疼痛。多伴有淋巴結(jié)、骨髓及外周血受累。病理學(xué)表現(xiàn)為表皮幾乎無浸潤，真皮及皮下脂肪組織可見彌漫的形態(tài)一致的母細(xì)胞樣細(xì)胞浸潤，該細(xì)胞中等大小，核大，不規(guī)則，可見Grens帶，無血管浸潤及壞死。免疫病理學(xué)染色結(jié)果在診斷BPDCN時尤為重要。主要表現(xiàn)為CD4(+)、CD31(+)、CD56(+)、 CD123(+)、 CD3(-)、 CD20(-)、 CD117(-)、 MPO(-)、EBER(-)。
[Abstract]:Blastic plasmacytoid dendritic cell tumor (blastic plasmacytoid dendriticcell neoplasm, BPDCN) is a rare hematopoietic malignancies, often with skin lesions as the main clinical manifestations, and lymph node, soft tissue, peripheral blood, bone marrow, even aggressive, central nervous system involvement with.BPDCN the higher the rapid progress of the diagnosis depends on the histopathology and immunohistochemical pathology results, and because the histopathological changes of nonspecific immune pathological examination, must be combined with the comprehensive, the exclusion of other similar hematopoietic system malignant tumor can be diagnosed. So the diagnosis is difficult, easy to be misdiagnosed. At present, the standard treatment there is no BPDCN scheme, the use of chemotherapy in acute leukemia. In the early stages of the disease, chemotherapy can achieve complete remission (completeremission, CR), but the majority of patients quickly relapsed. Studies have shown that made The therapeutic value of stem cell transplantation (SCT) is better and its therapeutic value is still being explored. The prognosis of this disease is poor.
Objective: to analyze the clinical manifestations, histopathological features and immuno pathological characteristics of mother cell plasma cell like dendritic cell tumors, so as to provide further clinical evidence for the correct diagnosis and differential diagnosis of this disease.
Methods: the clinical characteristics, histopathology and immunological pathology of 2 patients diagnosed as mother cell plasma cell like dendritic cell tumor were analyzed retrospectively.
Results: the clinical manifestations of common skin involvement, mostly purple papules, nodules can be isolated or multiple, occasional pruritus, pain, also visible lymph node, peripheral blood and bone marrow infiltration. The pathological findings: no infiltration of epidermal stem cell like tumor cells, dermal and subcutaneous adipose tissue showed diffuse infiltration of the cell morphology is uniform, medium size, thin cytoplasm, large nuclei, oval or irregular, between the epidermis and dermis showed no cell infiltration zone (Grens zone), no vascular infiltration and necrosis. Immunopathology: CD4, CD56, CD123, CD31, CD43, CD3, CD20, regular expression, CD34 CD117, MPO, and EBER were all negative. Treatment and prognosis: there is no standard treatment for the disease, rapid progress, disease worsening, disease stage, chemotherapy is effective, but easy to relapse, poor prognosis.
Conclusion: blastic plasmacytoid dendritic cell tumor in the elderly. Skin is blue purple papules, nodules, occasional pruritus, pain. Lymph node, peripheral blood and bone marrow involvement. Pathology showed almost no epidermal infiltration, the same shape of oocyte like cells in dermis and subcutaneous fat the organization showed diffuse infiltration of the cells of medium size, large nuclei and irregular, with no visible Grens, vascular invasion and necrosis. The immunohistochemistry staining results is very important in the diagnosis of BPDCN. The CD4 (+), CD31 (+), CD56 (+), CD123 (+), CD3 (-), CD20 (-), CD117 (-), MPO (-), EBER (-).

【學(xué)位授予單位】：吉林大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R739.5

【參考文獻】

相關(guān)期刊論文 前1條

1 何苗;肖林;夏云飛;林遠(yuǎn)雄;;母細(xì)胞性漿細(xì)胞樣樹突細(xì)胞腫瘤一例報告及文獻復(fù)習(xí)[J];中華腫瘤防治雜志;2012年20期

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本文編號：1377794