20例Castleman病的臨床與病理特征分析
發(fā)布時(shí)間：2019-06-20 01:25
【摘要】：目的:分析總結(jié)20例Castleman病(CD)患者的臨床表現(xiàn)、病理特點(diǎn)、治療方法及預(yù)后,以提高對(duì)CD的認(rèn)識(shí)。方法:選取南京大學(xué)附屬金陵醫(yī)院自2010年2月至2017年4月收治的20例Castleman病患者,回顧性分析其臨床特征、組織病理學(xué)改變、治療方法及預(yù)后。結(jié)果:①20例患者均行淋巴結(jié)活檢明確診斷為CD。②20例CD患者中,男11例、女9例,年齡14至73歲,中位年齡41.5歲,UCD9例,MCD11例,HV型12例,PC型8例。③9例UCD患者,透明血管型8例,漿細(xì)胞型1例,臨床主要表現(xiàn)為局部淋巴結(jié)腫大,3例累及肺門、2例累及縱隔、4例累及腹膜后,主要有局部壓迫癥狀如胸痛、胸悶、腹痛、腹脹等,余基本無(wú)全身癥狀及多系統(tǒng)受累表現(xiàn)。9例UCD患者中8例實(shí)驗(yàn)室檢查無(wú)明顯異常,僅1例合并有自身免疫性溶血性貧血。④11例MCD患者中漿細(xì)胞型7例,透明血管型4例,臨床表現(xiàn)為全身淋巴結(jié)腫大,多數(shù)累及頸部、腋窩、縱膈、腹股溝、腸系膜等處,9例患者多伴有全身和多系統(tǒng)表現(xiàn),包括3例肝脾腫大,4例漿膜腔積液,5例腎受累,1例骨髓細(xì)胞學(xué)結(jié)果顯示嗜酸粒細(xì)胞增多性骨髓象,1例合并過(guò)敏性肉芽腫性血管炎,1例合并POEMS綜合征。11例MCD患者中,7例貧血,8例CRP升高,8例低蛋白血癥,5例血沉升高,2例患者檢測(cè)血清IL-6均增高,1例免疫固定電泳圖譜見入型單克隆IgA和游離λ鏈。6例行骨髓細(xì)胞學(xué)檢查中,5例正常,1例嗜酸細(xì)胞增高。8例患者行抗核抗體譜和抗中性粒細(xì)胞細(xì)胞質(zhì)抗體(ANCA)檢測(cè),4例陽(yáng)性,其中2例表現(xiàn)為抗核抗體(ANA)陽(yáng)性、1例為抗SSA抗體陽(yáng)性、1例ANA及ANCA均陽(yáng)性。⑤MCD中有5例累及腎臟,其中男4例、女1例,中位年齡42歲,均表現(xiàn)為蛋白尿陽(yáng)性及腎功能受損。3例PC型、2例HV型。2例患者行腎活檢,病理改變分別為腎小球膜增殖樣病變及AA型腎淀粉樣變性。⑥20例患者行CT顯示病灶多呈界限清楚的圓形、類圓形軟組織密度影,密度較均勻,其中3例病例可在病灶內(nèi)部鈣化影,3例患者行PET-CT示多發(fā)腫大淋巴結(jié)氟代脫氧葡萄糖代謝輕度增高。⑦9例UCD患者中8例行手術(shù)切除病灶,1例行放化療綜合治療;8例痊愈,1例患者失訪。11例MCD患者中,9例患者分別予糖皮質(zhì)激素單藥、CHOP、CTD、VRD等化療方案治療,2例完全緩解、2例部分緩解、4例疾病進(jìn)展、1例死亡;2例行腹膜后占位切除術(shù)后分別予糖皮質(zhì)激素單藥和CHOP化療,2例患者疾病進(jìn)展。結(jié)論:CD確診主要依靠病理檢查,CD可發(fā)生于任何年齡段,男女發(fā)病率基本一致,且多無(wú)特異性臨床表現(xiàn)及影像學(xué)表現(xiàn),不同臨床分型,臨床表現(xiàn)有很大差異。UCD患者常無(wú)明顯臨床癥狀,多表現(xiàn)為單一部位淋巴結(jié)腫大,實(shí)驗(yàn)室檢查多正常,手術(shù)切除是其主要治療方式,且預(yù)后較好。MCD患者常表現(xiàn)為全身多部位淋巴結(jié)腫大,臨床癥狀復(fù)雜,可累及多臟器、多系統(tǒng),其中包括腎臟,導(dǎo)致不同腎臟病理類型,需要綜合治療。
[Abstract]:Objective: to analyze and summarize the clinical manifestations, pathological features, treatment and prognosis of 20 patients with (CD) with Castleman's disease in order to improve the understanding of CD. Methods: from February 2010 to April 2017, 20 patients with Castleman disease were selected from Jinling Hospital affiliated to Nanjing University. The clinical features, histopathological changes, treatment and prognosis were analyzed retrospectively. Results: all the 120 patients with CD were diagnosed by lymph node biopsy. there were 11 males and 9 females, aged 14 to 73 years, median age 41.5 years, UCD9, MCD11, HV 12, PC 8. 39 patients with UCD, 8 patients with hyaluronic vessel type, 1 patient with plasma cell type, 3 patients with local lymphadenopathy, 3 patients with hilar involvement, 2 patients with mediastinum, 4 patients with retroperitoneal involvement. There were mainly symptoms of local compression, such as chest pain, chest tightness, abdominal pain, abdominal distension, etc. 8 of 9 patients with UCD had no obvious abnormalities in laboratory examination, only 1 patient had autoimmune hemolytic anemia. In 411 patients with MCD, plasma cell type was found in 7 patients, hyaluronic vessel type in 4 patients, and the clinical manifestations were enlarged lymph nodes, mostly involving neck, axillary, mediastinum and groin. In the mesentery, 9 patients were accompanied by systemic and multisystematic manifestations, including 3 cases of hepatosplenomegaly, 4 cases of serous effusion, 5 cases of renal involvement, 1 case of bone marrow Cytology, 1 case of allergic granulomatous vasculitis, 1 case of POEMS syndrome. In 11 cases of MCD, 7 cases were anemia, 8 cases were CRP elevation, 8 cases were hypoproteemia, 5 cases were erythrocyte sedimentation rate. Serum IL-6 was increased in 2 patients, and monoclonal IgA and free 位 chain were found in 1 case of immunofixation electrophoresis. In 6 cases, 5 cases were normal and 1 case was increased in eosinophils. 8 cases were detected by antinuclear antibody spectrum and antinephrine cytoplasmic antibody (ANCA), 4 cases were positive, 2 cases were positive for antinuclear antibody (ANA) and 1 case was positive for anti-SSA antibody. Both ANA and ANCA were positive in 1 case. 5 cases of 5MCD involved the kidney, including 4 males and 1 female, with a median age of 42 years. 3 cases were PC type and 2 cases were HV type. 2 cases underwent renal biopsy. the pathological changes were bulbar membrane proliferating lesion and AA type renal amyloidosis, respectively. CT showed that most of the lesions were round and round, similar to round soft tissue density. The density was uniform, 3 cases were calcified in the lesion, 3 cases were slightly increased in fluorodeoxyglucose metabolism of multiple enlarged lymph nodes by PET-CT. Among 79 cases of UCD, 8 cases underwent surgical resection of the lesion and 1 case was treated with radiotherapy and chemotherapy. Among 11 patients with MCD, 9 patients were treated with glucocorticoid single drug, CHOP,CTD,VRD and other chemotherapy regimen, 2 cases were completely relieved, 2 cases were partially relieved, 4 cases were disease progress, 1 case died, 2 cases were treated with glucocorticoid single drug and CHOP chemotherapy respectively, and 2 cases were treated with glucocorticoid chemotherapy and CHOP chemotherapy respectively after retroperitoneal space occupying resection, 2 cases were completely relieved, 2 cases were partial remission, 4 cases were disease progress, 2 cases were treated with glucocorticoid monotherapy and CHOP chemotherapy, 2 cases were treated with glucocorticoid monotherapy and CHOP chemotherapy respectively. Conclusion: the diagnosis of CD mainly depends on pathological examination. CD can occur in any age group, and the incidence of UCD is basically the same in men and women, and there are many non-specific clinical and imaging manifestations, and there are great differences in clinical manifestations. UCD patients often have no obvious clinical symptoms, most of them are single site lymph node enlargement, most of them are normal in laboratory examination, and surgical resection is the main treatment. The prognosis of MCD patients is good. MCD patients often show multiple lymph node enlargement, complex clinical symptoms, can involve multiple organs, multiple systems, including kidney, resulting in different renal pathological types, need comprehensive treatment.
【學(xué)位授予單位】：南京大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2018
【分類號(hào)】：R551.2
本文編號(hào)：2502799
[Abstract]:Objective: to analyze and summarize the clinical manifestations, pathological features, treatment and prognosis of 20 patients with (CD) with Castleman's disease in order to improve the understanding of CD. Methods: from February 2010 to April 2017, 20 patients with Castleman disease were selected from Jinling Hospital affiliated to Nanjing University. The clinical features, histopathological changes, treatment and prognosis were analyzed retrospectively. Results: all the 120 patients with CD were diagnosed by lymph node biopsy. there were 11 males and 9 females, aged 14 to 73 years, median age 41.5 years, UCD9, MCD11, HV 12, PC 8. 39 patients with UCD, 8 patients with hyaluronic vessel type, 1 patient with plasma cell type, 3 patients with local lymphadenopathy, 3 patients with hilar involvement, 2 patients with mediastinum, 4 patients with retroperitoneal involvement. There were mainly symptoms of local compression, such as chest pain, chest tightness, abdominal pain, abdominal distension, etc. 8 of 9 patients with UCD had no obvious abnormalities in laboratory examination, only 1 patient had autoimmune hemolytic anemia. In 411 patients with MCD, plasma cell type was found in 7 patients, hyaluronic vessel type in 4 patients, and the clinical manifestations were enlarged lymph nodes, mostly involving neck, axillary, mediastinum and groin. In the mesentery, 9 patients were accompanied by systemic and multisystematic manifestations, including 3 cases of hepatosplenomegaly, 4 cases of serous effusion, 5 cases of renal involvement, 1 case of bone marrow Cytology, 1 case of allergic granulomatous vasculitis, 1 case of POEMS syndrome. In 11 cases of MCD, 7 cases were anemia, 8 cases were CRP elevation, 8 cases were hypoproteemia, 5 cases were erythrocyte sedimentation rate. Serum IL-6 was increased in 2 patients, and monoclonal IgA and free 位 chain were found in 1 case of immunofixation electrophoresis. In 6 cases, 5 cases were normal and 1 case was increased in eosinophils. 8 cases were detected by antinuclear antibody spectrum and antinephrine cytoplasmic antibody (ANCA), 4 cases were positive, 2 cases were positive for antinuclear antibody (ANA) and 1 case was positive for anti-SSA antibody. Both ANA and ANCA were positive in 1 case. 5 cases of 5MCD involved the kidney, including 4 males and 1 female, with a median age of 42 years. 3 cases were PC type and 2 cases were HV type. 2 cases underwent renal biopsy. the pathological changes were bulbar membrane proliferating lesion and AA type renal amyloidosis, respectively. CT showed that most of the lesions were round and round, similar to round soft tissue density. The density was uniform, 3 cases were calcified in the lesion, 3 cases were slightly increased in fluorodeoxyglucose metabolism of multiple enlarged lymph nodes by PET-CT. Among 79 cases of UCD, 8 cases underwent surgical resection of the lesion and 1 case was treated with radiotherapy and chemotherapy. Among 11 patients with MCD, 9 patients were treated with glucocorticoid single drug, CHOP,CTD,VRD and other chemotherapy regimen, 2 cases were completely relieved, 2 cases were partially relieved, 4 cases were disease progress, 1 case died, 2 cases were treated with glucocorticoid single drug and CHOP chemotherapy respectively, and 2 cases were treated with glucocorticoid chemotherapy and CHOP chemotherapy respectively after retroperitoneal space occupying resection, 2 cases were completely relieved, 2 cases were partial remission, 4 cases were disease progress, 2 cases were treated with glucocorticoid monotherapy and CHOP chemotherapy, 2 cases were treated with glucocorticoid monotherapy and CHOP chemotherapy respectively. Conclusion: the diagnosis of CD mainly depends on pathological examination. CD can occur in any age group, and the incidence of UCD is basically the same in men and women, and there are many non-specific clinical and imaging manifestations, and there are great differences in clinical manifestations. UCD patients often have no obvious clinical symptoms, most of them are single site lymph node enlargement, most of them are normal in laboratory examination, and surgical resection is the main treatment. The prognosis of MCD patients is good. MCD patients often show multiple lymph node enlargement, complex clinical symptoms, can involve multiple organs, multiple systems, including kidney, resulting in different renal pathological types, need comprehensive treatment.
【學(xué)位授予單位】：南京大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2018
【分類號(hào)】：R551.2
【參考文獻(xiàn)】
相關(guān)期刊論文 前2條
1 高然;張蕊;曲藝;李艷;;Castleman病82例臨床特征分析[J];現(xiàn)代腫瘤醫(yī)學(xué);2013年08期
2 劉寧;邱法波;李奉達(dá);;Castleman's病流行病學(xué)及臨床特征[J];世界華人消化雜志;2008年30期
，本文編號(hào)：2502799
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