Itami M, Teshima S, Asakuma Y, Chino H, Aoyama K, Fukushima N
Department of Pathology, Fraternity Memorial Hospital, Tokyo, Japan.
Acta Cytol. 1997 Mar-Apr;41(2):522-8. doi: 10.1159/000332550.
Pulmonary lymphangiomyomatosis (P-LAM) is a rare disease, characterized by an abnormal proliferation of smooth muscle throughout the lung and occurring in females of reproductive age. The typical clinical picture in P-LAM is progressive dyspnea, often punctuated by episodes of chylothorax or pneumothorax, with eventual death from respiratory failure. The definitive diagnosis is usually performed on open lung biopsies.
The cytologic findings in a chylous pleural effusion from a patient with P-LAM are presented. The effusion contained an abundance of globular cell clusters composed of two distinct cell types, inner core spindle cells and surface flat cells. Immunocytochemical examination revealed that the core cells were derived from immature smooth muscle cells and the surface cells from endothelium. Histologic examination of the excised specimen showed the typical findings of P-LAM, and the dilated peripheral lymphatics contained cell clusters similar to those in the pleural effusion. The cell clusters in the lymphatics in the P-LAM lung tissue appeared to have ruptured into the thoracic cavity.
Cell clusters seem to be pathognomonic of P-LAM, and cytologic examination can obviate the need to perform open lung biopsy to confirm the diagnosis.
肺淋巴管平滑肌瘤病（P-LAM）是一种罕见疾病，其特征为全肺平滑肌异常增殖，好发于育龄期女性。P-LAM的典型临床表现为进行性呼吸困难，常伴有乳糜胸或气胸发作，最终死于呼吸衰竭。确诊通常依靠开胸肺活检。
本文报道了1例P-LAM患者乳糜性胸腔积液的细胞学检查结果。胸腔积液中含有大量由两种不同细胞类型组成的球状细胞簇，即内层梭形细胞和表面扁平细胞。免疫细胞化学检查显示，核心细胞来源于未成熟平滑肌细胞，表面细胞来源于内皮细胞。切除标本的组织学检查显示出P-LAM的典型表现，扩张的外周淋巴管中含有与胸腔积液中相似的细胞簇。P-LAM肺组织淋巴管中的细胞簇似乎已破裂进入胸腔。
细胞簇似乎是P-LAM的特征性表现，细胞学检查可避免进行开胸肺活检来确诊。
