本文選題：皮膚淋巴瘤　切入點：臨床表現(xiàn)　出處：《昆明醫(yī)科大學》2017年碩士論文　論文類型：學位論文

【摘要】：[目的]1. 了解云南地區(qū)原發(fā)性皮膚淋巴瘤流行病學特征;2.分析總結(jié)原發(fā)性皮膚淋巴瘤的臨床與病理特征,深入對原發(fā)皮膚淋巴瘤的認識;3.對各型原發(fā)性皮膚淋巴瘤臨床與組織病理特征進行分析、比較、鑒別,以期提高早期診斷符合率,早期治療,改善預后。[方法]對2006年1月至2016年12月在昆明醫(yī)科大學第一附屬醫(yī)院皮膚科經(jīng)臨床、病理確診為原發(fā)性皮膚淋巴瘤的72例病例進行回顧性調(diào)查分析。結(jié)合臨床及病理資料進行分類,歸納不同類型原發(fā)性皮膚淋巴瘤的臨床與病理特征。[結(jié)果]1.在原發(fā)性皮膚淋巴瘤(PCL)中,以T細胞淋巴瘤(PCTCL)多見,占86.11%,其中蕈樣肉芽腫(MF)占55.56%; B細胞淋巴瘤(PCBCL)較少見,占13.89%。2.PCL中男女比例1.4:1,發(fā)病年齡3～83歲,平均發(fā)病年齡46.49±19.95歲(中位年齡49歲),高峰年齡階段為32～61歲。3.本組72例PCL病程最短0.5月,最長30年,平均確診前病程41.27月。MF平均病程最長(55.98月),PCTCL與PCBCL確診前病程的分布差異有統(tǒng)計學意義(P=0.0110.05)。4.多發(fā)皮損占86.11%,單發(fā)皮損占13.89%,PCBCL單發(fā)率(50%)明顯高于PCTCL(8.06%);皮損涉及全身各個部位,泛發(fā)者最為多見,占45.83%;其次為軀干,占22.22%;四肢占16.67%;頭面頸部最少,占15.28%。5.48.61%的患者伴有不同程度瘙癢,19.44%患者伴有疼痛,31.94%無明顯自覺癥狀。PCBCL無自覺癥狀者(80%)明顯高于PCTCL (24.19%)。6.PCTCL臨床與病理特征:萆樣肉芽腫(MF)最常見,占PCL55.56%,早期皮疹及組織病理缺乏特異性,組織學特征為淋巴細胞親表皮性、Pautrier微膿瘍、真皮內(nèi)腦回狀細胞核的T淋巴細胞不同程度的浸潤。皮膚CD30+T細胞淋巴增生性疾病臨床表現(xiàn)多樣,潰瘍易見,部分皮損可自然消退,組織學特征為真皮內(nèi)致密的細胞浸潤,細胞核大、胞漿豐富,大部分瘤細胞CD30陽性。皮下脂膜炎樣T細胞淋巴瘤(SPTCL)臨床以四肢、軀干孤立性或多發(fā)性皮下結(jié)節(jié)或斑塊為主,常伴有發(fā)熱,全血細胞減少癥狀,組織學特征為皮下脂肪組織多形性淋巴細胞浸潤,瘤細胞圍繞脂肪細胞呈花邊狀排列,瘤細胞表達細胞毒T細胞的免疫表型。種痘水皰病樣淋巴瘤好發(fā)于兒童,臨床表現(xiàn)與HV類似,組織病理表現(xiàn)為以小-中等大小的多形性淋巴樣細胞呈血管中心性浸潤,腫瘤細胞表達細胞毒性T細胞標志物和細胞毒顆粒相關(guān)蛋白,部分病例NK細胞相關(guān)抗原CD56陽性,EBER 一般為陽性。結(jié)外鼻型NK/T細胞淋巴瘤的臨床表現(xiàn)主要為鼻或者面中部的進行性破壞性病變,病理特征為異型淋巴細胞血管中心性浸潤,NK細胞標志物、細胞毒顆粒相關(guān)蛋白及EBER陽性。7. PCBCL臨床表現(xiàn)多為特異性的單發(fā)或多發(fā)的皮膚結(jié)節(jié)或腫物,無或僅有輕微自覺癥狀。組織病理上表皮很少受累,真皮淺層有無浸潤帶,真皮及皮下組織見致密的淋巴細胞浸潤。免疫組化B細胞標記物(CD20、CD79a等)陽性。[結(jié)論]1.在PCL發(fā)病情況中,皮膚T細胞淋巴瘤(PCTCL)多見,占86.11%,其中蕈樣肉芽腫(MF)占55.56%;皮膚B細胞淋巴瘤(PCBCL)較少見,占13.89%。2. PCL可發(fā)病于任何年齡,以中老年人群好發(fā);皮損多為泛發(fā)性,以軀干、四肢為主;除部分MF痛癢劇烈以外,多無明顯自覺癥狀或僅輕微瘙癢。3. PCL分類復雜,各型臨床表現(xiàn)與組織病理有特征性表現(xiàn),PCBCL皮疹較PCTCL局限,潰瘍少見,組織病理浸潤深,很少侵犯表皮;PCTCL親表皮現(xiàn)象常見,浸潤較淺。4. MF是PCL最常見的類型,早期臨床表現(xiàn)及組織病理缺乏特異性,容易誤診,需加強臨床觀察和組織病理隨訪。5.臨床表現(xiàn)、組織病理特征、免疫組化和基因重排檢測技術(shù)的結(jié)合,對PCL的診斷和具體分型至關(guān)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.

【學位授予單位】：昆明醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R739.5

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