中华结核和呼吸杂志
中華結覈和呼吸雜誌
중화결핵화호흡잡지
Chinese Journal of Tuberculosis and Respiratory Diseases
2014年
5期
337-342
,共6页
田欣伦%葛莉%冯瑞娥%柳涛%黄慧%徐作军%许文兵%刘鸿瑞
田訢倫%葛莉%馮瑞娥%柳濤%黃慧%徐作軍%許文兵%劉鴻瑞
전흔륜%갈리%풍서아%류도%황혜%서작군%허문병%류홍서
巨淋巴结增生%肺%淋巴结
巨淋巴結增生%肺%淋巴結
거림파결증생%폐%림파결
Giant lymph node hyperplasia%Pulmonary%Lymph nodes
目的 探讨发生于肺实质的多中心性巨淋巴结增生(Castleman病)的临床及病理特点.方法 回顾性总结了2008年7月至2013年4月北京协和医院住院及会诊、且经外科肺活检病理诊断的6例发生于肺实质的多中心性巨淋巴结增生患者的临床、影像及病理资料,并复习相关文献.活检标本经4％中性甲醛固定,常规石蜡切片,HE及免疫组织化学染色,3例行B和T淋巴细胞基因重排检测.结果 6例均为女性,年龄31 ～ 68岁,中位年龄为49.5岁.临床表现主要为发热(4例)、咳嗽(3例)及淋巴结肿大(6例)等.实验室检查:ESR为38 ～ 120 mm/1 h(5例),C反应蛋白为23.2 ～1 610 mg/L(4例),丙种球蛋白为27.3％ ～31.5％(2例).胸部CT显示沿淋巴管分布的多发性肺实变结节、磨玻璃影和小叶间隔增宽,伴纵隔多发性淋巴结肿大.病理检查5例为浆细胞型,1例为透明血管型,6例病变区均可见残存的肺泡结构,提示其并非肺内淋巴结的巨淋巴结增生.浆细胞型主要表现为肺间质内大量成熟的浆细胞浸润;透明血管型则以生发中心萎缩和滤泡外套区淋巴细胞呈同心层状排列为特点.B和T淋巴细胞基因重排检测均为阴性.随访2 ～ 60个月(平均31个月),2例浆细胞型行CHOP方案(环磷酰胺+多柔比星+长春新碱+泼尼松)化疗,其余3例未进行任何治疗,病情平稳存活;1例透明血管型行CHOP方案化疗,曾有一过性好转,但于诊断后7个月死于疾病恶化.结论 胸部的巨淋巴结增生常发生于纵隔及肺门或肺内淋巴结,发生于肺实质的巨淋巴结增生罕见,常见于中老年女性,临床表现有呼吸系统症状,并常伴有全身症状,如发热、ESR增快和高丙种球蛋白血症等,胸部CT常表现为沿淋巴管分布的肺内多发性结节及磨玻璃影,常伴纵隔淋巴结肿大.病理组织学改变与淋巴结巨淋巴结增生组织形态特点相似,不同于淋巴细胞性间质性肺炎,免疫组织化学及基因重排检测有助于与其他肺部淋巴组织增生性疾病鉴别.
目的 探討髮生于肺實質的多中心性巨淋巴結增生(Castleman病)的臨床及病理特點.方法 迴顧性總結瞭2008年7月至2013年4月北京協和醫院住院及會診、且經外科肺活檢病理診斷的6例髮生于肺實質的多中心性巨淋巴結增生患者的臨床、影像及病理資料,併複習相關文獻.活檢標本經4％中性甲醛固定,常規石蠟切片,HE及免疫組織化學染色,3例行B和T淋巴細胞基因重排檢測.結果 6例均為女性,年齡31 ～ 68歲,中位年齡為49.5歲.臨床錶現主要為髮熱(4例)、咳嗽(3例)及淋巴結腫大(6例)等.實驗室檢查:ESR為38 ～ 120 mm/1 h(5例),C反應蛋白為23.2 ～1 610 mg/L(4例),丙種毬蛋白為27.3％ ～31.5％(2例).胸部CT顯示沿淋巴管分佈的多髮性肺實變結節、磨玻璃影和小葉間隔增寬,伴縱隔多髮性淋巴結腫大.病理檢查5例為漿細胞型,1例為透明血管型,6例病變區均可見殘存的肺泡結構,提示其併非肺內淋巴結的巨淋巴結增生.漿細胞型主要錶現為肺間質內大量成熟的漿細胞浸潤;透明血管型則以生髮中心萎縮和濾泡外套區淋巴細胞呈同心層狀排列為特點.B和T淋巴細胞基因重排檢測均為陰性.隨訪2 ～ 60箇月(平均31箇月),2例漿細胞型行CHOP方案(環燐酰胺+多柔比星+長春新堿+潑尼鬆)化療,其餘3例未進行任何治療,病情平穩存活;1例透明血管型行CHOP方案化療,曾有一過性好轉,但于診斷後7箇月死于疾病噁化.結論 胸部的巨淋巴結增生常髮生于縱隔及肺門或肺內淋巴結,髮生于肺實質的巨淋巴結增生罕見,常見于中老年女性,臨床錶現有呼吸繫統癥狀,併常伴有全身癥狀,如髮熱、ESR增快和高丙種毬蛋白血癥等,胸部CT常錶現為沿淋巴管分佈的肺內多髮性結節及磨玻璃影,常伴縱隔淋巴結腫大.病理組織學改變與淋巴結巨淋巴結增生組織形態特點相似,不同于淋巴細胞性間質性肺炎,免疫組織化學及基因重排檢測有助于與其他肺部淋巴組織增生性疾病鑒彆.
목적 탐토발생우폐실질적다중심성거림파결증생(Castleman병)적림상급병리특점.방법 회고성총결료2008년7월지2013년4월북경협화의원주원급회진、차경외과폐활검병리진단적6례발생우폐실질적다중심성거림파결증생환자적림상、영상급병리자료,병복습상관문헌.활검표본경4％중성갑철고정,상규석사절편,HE급면역조직화학염색,3례행B화T림파세포기인중배검측.결과 6례균위녀성,년령31 ～ 68세,중위년령위49.5세.림상표현주요위발열(4례)、해수(3례)급림파결종대(6례)등.실험실검사:ESR위38 ～ 120 mm/1 h(5례),C반응단백위23.2 ～1 610 mg/L(4례),병충구단백위27.3％ ～31.5％(2례).흉부CT현시연림파관분포적다발성폐실변결절、마파리영화소협간격증관,반종격다발성림파결종대.병리검사5례위장세포형,1례위투명혈관형,6례병변구균가견잔존적폐포결구,제시기병비폐내림파결적거림파결증생.장세포형주요표현위폐간질내대량성숙적장세포침윤;투명혈관형칙이생발중심위축화려포외투구림파세포정동심층상배렬위특점.B화T림파세포기인중배검측균위음성.수방2 ～ 60개월(평균31개월),2례장세포형행CHOP방안(배린선알+다유비성+장춘신감+발니송)화료,기여3례미진행임하치료,병정평은존활;1례투명혈관형행CHOP방안화료,증유일과성호전,단우진단후7개월사우질병악화.결론 흉부적거림파결증생상발생우종격급폐문혹폐내림파결,발생우폐실질적거림파결증생한견,상견우중노년녀성,림상표현유호흡계통증상,병상반유전신증상,여발열、ESR증쾌화고병충구단백혈증등,흉부CT상표현위연림파관분포적폐내다발성결절급마파리영,상반종격림파결종대.병리조직학개변여림파결거림파결증생조직형태특점상사,불동우림파세포성간질성폐염,면역조직화학급기인중배검측유조우여기타폐부림파조직증생성질병감별.
Objective To observe the clinicopathological features of pulmonary parenchymal involvement of muhicentric Castleman's disease(MCD).Methods Retrospective analysis was carried out for 6 patients of MCD with pulmonary parenchymal involvement who had been admitted to Peking Union Medical College Hospital from July 2008 to March 2013.Relevant literatures were reviewed.The diagnosis was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin.Sections were cut for HE and immunohistochemical stain.B cell and T cell gene rearrangement were tested in 3 cases.Results These 6 patients (all females) aged 31-68 years,with a median of 49.5years.The presenting symptoms were fever (4/6),cough (3/6),and lymphadenopathy (6/6).Laboratory study showed elevated ESR (5/6) and CRP (4/6),and hypergammaglobulinaemia (2/6).Chest CT showed multiple nodules with perilymphatic distribution and ground-glass opacity (GGO).Pathologically,there were 5 cases of plasma cell type and 1 case of hyaline vascular type.The plasma cell variant showed dense mature plasma cell infiltration in pulmonary interstitium.The hyaline vascular variant was characterized by the presence of regressed germinal centers and broad concentric mantle zones.The gene arrangement tests were all negative.During the follow-up period (range:2-60 months; mean:31 months),2 cases with plasma cell type received CHOP chemotherapy and then remained stable.One case with hyaline vascular type received CHOP chemotherapy but died due to deterioration of the disease.Conclusions In the thorax,Castleman' s disease usually manifests as hilar and mediastinal lymph node enlargement.Pulmonary parenchymal involvement by MCD is very rare.It is mostly seen in the elderly female,and can manifest with systemic symptoms.Chest CT usually reveals multiple nodules and GGO.It shows similar morphological characteristics to those found in lymph nodes.Immunohistochemistry and gene rearrangement test can help to differentiate it from other pulmonary lymphoproliferative diseases.
