From the Department of Pathology, University of Colorado, Aurora.
Arch Pathol Lab Med. 2015 Feb;139(2):274-7. doi: 10.5858/arpa.2013-0500-RS.
Pulmonary capillary hemangiomatosis is a rare cause of pulmonary hypertension characterized by extensive proliferation of pulmonary capillaries within alveolar septae. Clinical presentation is nonspecific and includes dyspnea, cough, chest pain, and fatigue. Radiology shows diffuse centrilobular ground-glass opacities. Pulmonary capillary hemangiomatosis is clinically and radiographically indistinguishable from peripheral venoocclusive disease, making microscopic diagnosis essential. Histologically, pulmonary capillary hemangiomatosis shows an abnormal proliferation of small, thin-walled capillaries that expand the alveolar septae. The endothelial cells that comprise these lesions are cytologically bland and show no mitotic activity. Pulmonary capillary hemangiomatosis is important to recognize because prostacyclin therapy, a mainstay in the treatment of pulmonary hypertension, has been reported to cause sudden respiratory distress and death in these patients. Prognosis of this disease remains poor, and the only definitive treatment is lung transplantation.
肺毛细血管血管瘤病是一种罕见的肺动脉高压病因，其特征是肺泡间隔内的肺毛细血管广泛增殖。临床表现无特异性，包括呼吸困难、咳嗽、胸痛和疲劳。放射学表现为弥漫性小叶中心磨玻璃影。肺毛细血管血管瘤病在临床和放射学上与外周静脉闭塞性疾病无法区分，因此显微镜下的诊断至关重要。组织学上，肺毛细血管血管瘤病表现为小而薄壁的毛细血管异常增殖，使肺泡间隔扩张。构成这些病变的内皮细胞细胞形态学上温和，无有丝分裂活性。肺毛细血管血管瘤病的认识非常重要，因为前列环素治疗是肺动脉高压治疗的主要方法，但据报道，这种治疗会导致这些患者突发呼吸窘迫和死亡。这种疾病的预后仍然很差，唯一明确的治疗方法是肺移植。
