北京大学学报(医学版)
北京大學學報(醫學版)
북경대학학보(의학판)
Journal of Peking University (Health Sciences)
2015年
5期
865-869
,共5页
熊焰%李晓霞%牟向东%李东%王颖%李挺
熊燄%李曉霞%牟嚮東%李東%王穎%李挺
웅염%리효하%모향동%리동%왕영%리정
肺毛细血管瘤病%肺静脉闭塞性疾病%肺动脉高压
肺毛細血管瘤病%肺靜脈閉塞性疾病%肺動脈高壓
폐모세혈관류병%폐정맥폐새성질병%폐동맥고압
Pulmonary capillary hemangiomatosis%Pulmonary veno-occlusive disease%Pulmonary arte-rial hypertension
报道1例肺毛细血管瘤病((pulmonary capillary hemangiomatosis,PCH),结合本例特点复习国内外文献报道和2008年WHO在Dana Point制定的肺高压临床分类,对该病的诊断、鉴别诊断以及病因和治疗进行介绍,提高各科医生对这一罕见病的认识。本例患者为中年女性,主要临床症状为活动后气促;右心导管检查示中度肺动脉高压;CT肺血管造影( CT pulmonary angiography , CTPA)示双肺弥漫磨玻璃密度小叶核心结节,肺动脉主干及分支增宽;组织病理学上表现为肺小动脉肌化,肌性肺动脉中膜增厚,肺泡壁毛细血管显著增生呈多排、结节状及片状;患者药物治疗无效,于3个月后死亡。 PCH是一种罕见、预后不良的血管病变,其诊断需要综合临床、影像及组织病理3方面的资料,组织病理学检查是最可靠的手段,对确诊具有决定性作用。该病在临床、影像及组织形态上均与肺静脉阻塞病( pulmonary veno-occlusive disease , PVOD)存在诸多相似和交叉之处,两者需要重点鉴别。在诸多病理学改变中,肺泡壁毛细血管增生是PCH最重要的特点,也是与PVOD相鉴别的关键点。肺移植被认为是PCH唯一有效的治疗手段,如果不及时进行,患者通常在确诊后几个月内死亡。
報道1例肺毛細血管瘤病((pulmonary capillary hemangiomatosis,PCH),結閤本例特點複習國內外文獻報道和2008年WHO在Dana Point製定的肺高壓臨床分類,對該病的診斷、鑒彆診斷以及病因和治療進行介紹,提高各科醫生對這一罕見病的認識。本例患者為中年女性,主要臨床癥狀為活動後氣促;右心導管檢查示中度肺動脈高壓;CT肺血管造影( CT pulmonary angiography , CTPA)示雙肺瀰漫磨玻璃密度小葉覈心結節,肺動脈主榦及分支增寬;組織病理學上錶現為肺小動脈肌化,肌性肺動脈中膜增厚,肺泡壁毛細血管顯著增生呈多排、結節狀及片狀;患者藥物治療無效,于3箇月後死亡。 PCH是一種罕見、預後不良的血管病變,其診斷需要綜閤臨床、影像及組織病理3方麵的資料,組織病理學檢查是最可靠的手段,對確診具有決定性作用。該病在臨床、影像及組織形態上均與肺靜脈阻塞病( pulmonary veno-occlusive disease , PVOD)存在諸多相似和交扠之處,兩者需要重點鑒彆。在諸多病理學改變中,肺泡壁毛細血管增生是PCH最重要的特點,也是與PVOD相鑒彆的關鍵點。肺移植被認為是PCH唯一有效的治療手段,如果不及時進行,患者通常在確診後幾箇月內死亡。
보도1례폐모세혈관류병((pulmonary capillary hemangiomatosis,PCH),결합본례특점복습국내외문헌보도화2008년WHO재Dana Point제정적폐고압림상분류,대해병적진단、감별진단이급병인화치료진행개소,제고각과의생대저일한견병적인식。본례환자위중년녀성,주요림상증상위활동후기촉;우심도관검사시중도폐동맥고압;CT폐혈관조영( CT pulmonary angiography , CTPA)시쌍폐미만마파리밀도소협핵심결절,폐동맥주간급분지증관;조직병이학상표현위폐소동맥기화,기성폐동맥중막증후,폐포벽모세혈관현저증생정다배、결절상급편상;환자약물치료무효,우3개월후사망。 PCH시일충한견、예후불량적혈관병변,기진단수요종합림상、영상급조직병리3방면적자료,조직병이학검사시최가고적수단,대학진구유결정성작용。해병재림상、영상급조직형태상균여폐정맥조새병( pulmonary veno-occlusive disease , PVOD)존재제다상사화교차지처,량자수요중점감별。재제다병이학개변중,폐포벽모세혈관증생시PCH최중요적특점,야시여PVOD상감별적관건점。폐이식피인위시PCH유일유효적치료수단,여과불급시진행,환자통상재학진후궤개월내사망。
We reported a case of pulmonary capillary hemangiomatosis (PCH) and introduced its diag-nosis, differential diagnosis, pathogenesis and development of treatment based on the review of Dana Point 2008 Classification of Pulmonary Hypertensiona and current literatures .A 43-year-old female presented progressive dyspnea, elevated pulmonary arterial pressures and CT pulmonary angiography (CTPA) imaging of main pulmonary arterial enlargement and wide spread ill -defined centrilobular nodules of ground-glass opacity.Her histologic features were proliferation of capillary channels within alveolar walls as well as muscularization of arterioles and medial hypertrophy of muscular pulmonary arteries.The treatment with diuretics and warfarin was used promptly , but unfortunately was ineffective. The patient died three months after diagnosis .PCH is a very rare vascular disease with poor prognosis . The diagnosis of PCH rests on the integration of clinical and radiographic information with pathologic fea -tures, however pathology is the most reliable means .Because clinical symptoms, imaging and histological features of pulmonary veno-occlusive disease (PVOD) and PCH broadly overlap, differential diagnosis should be made carefully.Among the various pathologic features proliferation of capillaries within alveolar walls is the key point for diagnosing PCH , which is also the most critical criteria for differentiating PCH from PVOD.So far the only definitive treatment for PCH is lung transplantation , without which the pa-tient will die several months after diagnosis .