母细胞性浆细胞样树突细胞肿瘤2例临床病理特征分析及文献回顾

发布时间：2018-01-04 08:18

本文关键词：母细胞性浆细胞样树突细胞肿瘤2例临床病理特征分析及文献回顾　出处：《吉林大学》2014年硕士论文　论文类型：学位论文

【摘要】：母细胞性浆细胞样树突细胞肿瘤（blastic plasmacytoid dendriticcell neoplasm，BPDCN）是一种较为罕见的淋巴造血系统恶性肿瘤，常以皮肤损害为主要临床表现，并可见淋巴结、软组织、外周血、骨髓、甚至中枢神经系统等侵犯。BPDCN具有较高的侵袭性，进展快，其诊断主要依据组织病理学及免疫病理学结果，又因其组织病理学改变缺少特异性，必须结合全面的免疫病理学检查，排除其他相似的造血系统恶性肿瘤方可诊断。所以诊断较难，易误诊。目前，尚无BPDCN的标准化治疗方案，多采用急性白血病的化疗方案。在疾病的初期，化疗可以达到完全缓解（completeremission，CR），但多数患者很快复发。有研究表明，造血干细胞移植（stem cell transplantation, SCT）疗效较好，其治疗价值仍在探究中。该病预后较差。目的：分析母细胞性浆细胞样树突细胞肿瘤的临床表现、组织病理学特点及免疫病理学特点，为该病正确诊断及鉴别诊断提供进一步临床依据。方法：分析2例明确诊断为母细胞性浆细胞样树突细胞肿瘤患者的临床特征，组织病理学及免疫病理学结果，回顾相关文献，分析并总结。结果：临床表现：常见皮肤受累，多为青紫色丘疹、结节，可孤立或多发，偶有瘙痒、疼痛，还可见淋巴结、外周血及骨髓浸润。病理学表现：表皮未见浸润，真皮及皮下脂肪组织可见弥漫的母细胞样肿瘤细胞浸润，该细胞中等大小，形态较一致，胞质稀少，细胞核大，卵圆形或不规则，表皮与真皮之间可见明显的无细胞浸润带（Grens带），未见血管浸润及坏死。免疫病理学表现：CD4、CD56、CD123、CD31、CD43常表达，CD3、CD20、CD34、CD117、MPO、EBER均阴性。治疗及预后：尚无标准化治疗方案，该病进展较快，病情进行性恶化，疾病初期，化疗有效，但易复发，预后差。结论：母细胞性浆细胞样树突细胞肿瘤好发于中老年人。皮疹多为青紫色丘疹、结节，偶有瘙痒、疼痛。多伴有淋巴结、骨髓及外周血受累。病理学表现为表皮几乎无浸润，真皮及皮下脂肪组织可见弥漫的形态一致的母细胞样细胞浸润，该细胞中等大小，核大，不规则，可见Grens带，无血管浸润及坏死。免疫病理学染色结果在诊断BPDCN时尤为重要。主要表现为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 (-).

【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R739.5

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