Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis -: A clinicopathologic study of 35 cases
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis -: A clinicopathologic study of 35 cases
复制标题
DOI：
10.1097/01.pas.0000209834.69972.e5
复制
发表时间：
2006-07-01
影响因子：
5.6
通讯作者：
Nicholson, Andrew G.
中科院分区：
文献类型：
--
作者：
Lantuejoul, Sylvie;Sheppard, Mary N.;Nicholson, Andrew G.
肺静脉闭塞性疾病（PVOD）和肺毛细血管瘤病（PCH）是肺动脉高压的罕见原因，被认为是不同的实体。然而，他们的表现是相似的，都与不良的预后有关。因此，我们回顾了来自35例诊断为PVOD（n = 30; av.年龄34 y，范围4至68 y; 19 M：11 F）或PCH（n = 5，av.年龄42岁，范围从9个月到60岁; 3男：2女），以评估其相互关系。在24例（73%）诊断为PVOD的病例中发现PCH，表现为小静脉周围病灶或肺实质弥漫性受累。PVOD中观察到的其他特征包括动脉中膜肥大和/或内膜纤维化（88%）、含铁血黄素沉着症（79%）、小静脉炎（12%）、梗死（9%）、间质纤维化（有时为局部瘢痕）（48%）和轻度淋巴细胞浸润（67%）。PCH患者中4例PVOD有静脉和动脉改变。PCH的病例也都表现为轻度间质淋巴细胞浸润，但没有小静脉炎或梗死。CD 34免疫染色显示毛细血管增生，主要累及肺泡。但也见于支气管和肺血管壁内。我们的数据表明，在大多数情况下，PCH代表一个继发性血管增生过程引起的毛细血管后阻塞，而不是一个单独的疾病。静脉闭塞的原因尚未确定，但偶尔发现的静脉炎表明这在某些病例中对静脉损伤起作用。
Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension, regarded by some as distinct entities. However, their presentations are similar and both are associated with poor prognoses. We therefore reviewed 38 specimens [autopsies (n = 15), surgical biopsies (n = 15), explants (n = 7), and pneumonectomy (I case)] from 35 patients diagnosed as either PVOD (n = 30; av. age 34 y, range 4 to 68 y; 19M: I I F) or PCH (n = 5, av. age 42 y, ranging from 9 months to 60 years; 3M:2F) to assess their interrelationship. PCH was identified in 24 (73%) cases diagnosed as PVOD, either as perivenular foci or diffuse involvement of the pulmonary parenchyma. Other features seen in PVOD were arterial medial hypertrophy and/or intimal fibrosis (88%), hemosiderosis (79%), venulitis (12%), infarction (9%), interstitial fibrosis (sometimes as localized scars) (48%), and a mild lymphocytic infiltrate (67%). In cases diagnosed as PCH, 4 showed venous and arterial changes of PVOD. Cases with PCH also all showed a mild interstitial lymphocytic infiltrate but there was no venulitis or infarction. Capillary proliferation was particularly well demonstrated by CD34 immunostaining and predominantly involved the alveoli., but was also seen within walls of bronchi and pulmonary vessels. Our data suggest that in the majority of cases PCH represents a secondary angioproliferative process caused by postcapillary obstruction rather than a separate disease. The cause of the venous obliteration was not identified but the occasional identification of phlebitis suggests this plays a role in venous damage in some cases.