72例原发性皮肤淋巴瘤临床与病理分析

发布时间：2018-03-12 19:06

本文选题：皮肤淋巴瘤　切入点：临床表现　出处：《昆明医科大学》2017年硕士论文　论文类型：学位论文

【摘要】：[目的]1. 了解云南地区原发性皮肤淋巴瘤流行病学特征;2.分析总结原发性皮肤淋巴瘤的临床与病理特征,深入对原发皮肤淋巴瘤的认识;3.对各型原发性皮肤淋巴瘤临床与组织病理特征进行分析、比较、鉴别,以期提高早期诊断符合率,早期治疗,改善预后。[方法]对2006年1月至2016年12月在昆明医科大学第一附属医院皮肤科经临床、病理确诊为原发性皮肤淋巴瘤的72例病例进行回顾性调查分析。结合临床及病理资料进行分类,归纳不同类型原发性皮肤淋巴瘤的临床与病理特征。[结果]1.在原发性皮肤淋巴瘤(PCL)中,以T细胞淋巴瘤(PCTCL)多见,占86.11%,其中蕈样肉芽肿(MF)占55.56%; B细胞淋巴瘤(PCBCL)较少见,占13.89%。2.PCL中男女比例1.4:1,发病年龄3～83岁,平均发病年龄46.49±19.95岁(中位年龄49岁),高峰年龄阶段为32～61岁。3.本组72例PCL病程最短0.5月,最长30年,平均确诊前病程41.27月。MF平均病程最长(55.98月),PCTCL与PCBCL确诊前病程的分布差异有统计学意义(P=0.0110.05)。4.多发皮损占86.11%,单发皮损占13.89%,PCBCL单发率(50%)明显高于PCTCL(8.06%);皮损涉及全身各个部位,泛发者最为多见,占45.83%;其次为躯干,占22.22%;四肢占16.67%;头面颈部最少,占15.28%。5.48.61%的患者伴有不同程度瘙痒,19.44%患者伴有疼痛,31.94%无明显自觉症状。PCBCL无自觉症状者(80%)明显高于PCTCL (24.19%)。6.PCTCL临床与病理特征:萆样肉芽肿(MF)最常见,占PCL55.56%,早期皮疹及组织病理缺乏特异性,组织学特征为淋巴细胞亲表皮性、Pautrier微脓疡、真皮内脑回状细胞核的T淋巴细胞不同程度的浸润。皮肤CD30+T细胞淋巴增生性疾病临床表现多样,溃疡易见,部分皮损可自然消退,组织学特征为真皮内致密的细胞浸润,细胞核大、胞浆丰富,大部分瘤细胞CD30阳性。皮下脂膜炎样T细胞淋巴瘤(SPTCL)临床以四肢、躯干孤立性或多发性皮下结节或斑块为主,常伴有发热,全血细胞减少症状,组织学特征为皮下脂肪组织多形性淋巴细胞浸润,瘤细胞围绕脂肪细胞呈花边状排列,瘤细胞表达细胞毒T细胞的免疫表型。种痘水疱病样淋巴瘤好发于儿童,临床表现与HV类似,组织病理表现为以小-中等大小的多形性淋巴样细胞呈血管中心性浸润,肿瘤细胞表达细胞毒性T细胞标志物和细胞毒颗粒相关蛋白,部分病例NK细胞相关抗原CD56阳性,EBER 一般为阳性。结外鼻型NK/T细胞淋巴瘤的临床表现主要为鼻或者面中部的进行性破坏性病变,病理特征为异型淋巴细胞血管中心性浸润,NK细胞标志物、细胞毒颗粒相关蛋白及EBER阳性。7. PCBCL临床表现多为特异性的单发或多发的皮肤结节或肿物,无或仅有轻微自觉症状。组织病理上表皮很少受累,真皮浅层有无浸润带,真皮及皮下组织见致密的淋巴细胞浸润。免疫组化B细胞标记物(CD20、CD79a等)阳性。[结论]1.在PCL发病情况中,皮肤T细胞淋巴瘤(PCTCL)多见,占86.11%,其中蕈样肉芽肿(MF)占55.56%;皮肤B细胞淋巴瘤(PCBCL)较少见,占13.89%。2. PCL可发病于任何年龄,以中老年人群好发;皮损多为泛发性,以躯干、四肢为主;除部分MF痛痒剧烈以外,多无明显自觉症状或仅轻微瘙痒。3. PCL分类复杂,各型临床表现与组织病理有特征性表现,PCBCL皮疹较PCTCL局限,溃疡少见,组织病理浸润深,很少侵犯表皮;PCTCL亲表皮现象常见,浸润较浅。4. MF是PCL最常见的类型,早期临床表现及组织病理缺乏特异性,容易误诊,需加强临床观察和组织病理随访。5.临床表现、组织病理特征、免疫组化和基因重排检测技术的结合,对PCL的诊断和具体分型至关重要。
[Abstract]:Objective to understand the Yunnan region]1. primary cutaneous lymphoma epidemiology characteristics; clinical and pathological features of 2. primary cutaneous lymphoma analysis, in-depth understanding of primary cutaneous lymphoma; 3. of each type of primary cutaneous lymphoma clinical and pathological features of analysis, comparison, identification, in order to improve the early diagnosis rate of early treatment, improve the prognosis. Methods in January 2006 to December 2016 in the Department of Dermatology of the First Affiliated Hospital of Kunming Medical University by clinical, pathological diagnosis of 72 cases of primary cutaneous lymphoma were retrospectively investigated. Combined with the clinical and pathological data classification, clinical and pathological features. Results of different types of primary cutaneous lymphoma the]1. in primary cutaneous lymphoma (PCL), T cell lymphoma (PCTCL) rare, which accounted for 86.11%, mycosis fungoides (MF) B cell lymphoma (accounting for 55.56%; PCBCL) is rare, accounting for 13.89%.2.PCL 1.4:1 in the proportion of men and women, age of 3~83 years, the average age of 46.49 + 19.95 years (median age 49 years), the peak age was 32~61 years 72 cases of.3. PCL had the shortest duration of 0.5 months, the longest 30 years before diagnosis, the average duration of 41.27 months the average duration of.MF the longest (55.98 months), there were statistically significant differences in the distribution of PCTCL and PCBCL before diagnosis of disease (P=0.0110.05).4. multiple lesions single lesion accounted for 86.11%, accounting for 13.89%, PCBCL single rate (50%) was significantly higher than that of PCTCL (8.06%); the lesions involved in various parts of the body, the most common, accounting for 45.83%; followed by the torso, limbs accounted for 22.22%; 16.67%; head and neck at least, for patients with varying degrees of itching 15.28%.5.48.61%, 19.44% patients with pain and 31.94% asymptomatic.PCBCL symptoms (80%) Ming Xiangao PCTCL (24.19%):.6.PCTCL: clinical and pathological features Granuloma (MF) the most common, accounting for PCL55.56%, early rash and pathological lack of specific histological features of lymphocyte epidermotroism, Pautrier microabscess, dermal infiltrate gyriform nucleus of T lymphocytes in different degrees. The skin CD30+T cell lymphoproliferative disease clinical manifestation, ulcer is easy to see, part of the lesion can natural subsidise, histological features of infiltration in the dermis and dense nuclei, abundant cytoplasm, most of the tumor cells were CD30 positive. Subcutaneous panniculitis like T cell lymphoma (SPTCL) clinical in limbs, trunk solitary or multiple subcutaneous nodules or plaques, often accompanied by fever, pancytopenia symptoms. The histological features of subcutaneous adipose tissue infiltration of pleomorphic lymphocytes and tumor cells around the fat cells were lace like arrangement of tumor cells expressing cytotoxic T cell immunophenotype hydroa vacciniforme like lymphoma. Found in children, clinical manifestations and pathological manifestations of HV is similar to the small - medium sized pleomorphic lymphoid cells showed angiocentric infiltration of cytotoxic T cell markers and cytotoxic granule associated protein expression in tumor cells, NK cell related antigen CD56 positive cases, EBER was positive. The clinical manifestations. The external nasal type NK/T cell lymphoma mainly for nasal or midface of destructive lesions, pathological features of atypical lymphocytes angiocentric infiltration, NK cell markers, TIA-1 and EBER positive.7. PCBCL clinical manifestations were single specific or multiple skin nodules or masses, or not only mild symptoms. Pathological skin rarely involved, the superficial dermis without infiltration, dermis and subcutaneous tissue see dense lymphocytic infiltration. Immunohistochemistry B cell markers (CD20, CD79 A). Conclusion]1. positive in PCL incidence of cutaneous T cell lymphoma (PCTCL) rare, which accounted for 86.11%, mycosis fungoides (MF) accounted for 55.56%; cutaneous B cell lymphoma (PCBCL) is a rare, accounting for 13.89%.2. PCL can occur at any age in the elderly population, good hair skin; generalized, the trunk, limbs; in addition to part of the MF severe itching, no obvious symptoms or only mild pruritus.3. PCL classification is complex, various types of clinical manifestations and pathological features of PCBCL rash were the limitations of PCTCL, ulcer rare, pathological depth of invasion, little invasion of epidermis; PCTCL Pro a common phenomenon of skin, shallow invasion.4. MF PCL is the most common type, early clinical manifestations and pathological lack of specificity, easy to misdiagnosis, the need to strengthen the clinical manifestation and pathological observation were.5., pathological features, immunohistochemistry and gene rearrangement detection technology The combination is essential for the diagnosis and specific typing of PCL.

【学位授予单位】：昆明医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R739.5

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