Kinoshita Yoshiaki, Sakamoto Atsuhiko, Hidaka Kouko
Division of Respiratory Medicine, Department of Internal Medicine, National Hospital Organization, Kokura Medical Center, Kitakyushu, Japan.
BMJ Case Rep. 2014 Oct 17;2014:bcr2014205701. doi: 10.1136/bcr-2014-205701.
Chyloptysis and chylous pulmonary congestion are extremely rare complications of lymphangioleiomyomatosis (LAM). We report a case of a 50-year-old woman with tuberous sclerosis complex-associated LAM, who presented with expectorating milky-white bronchial casts. She was diagnosed with chyloptysis and chylous pulmonary congestion by sputum analysis. Her symptoms and lung infiltration were improved by oral sirolimus therapy; moreover, serum Krebs von den Lungen-6 (KL-6) levels paralleled the symptoms and lung infiltration of these complications. We suggest that serum KL-6 may be a useful monitoring biomarker of chyloptysis and chylous pulmonary congestion in LAM.
乳糜痰和乳糜性肺充血是淋巴管平滑肌瘤病（LAM）极为罕见的并发症。我们报告一例50岁患有结节性硬化症相关LAM的女性患者，该患者咳出乳白色支气管管型。通过痰液分析，她被诊断为乳糜痰和乳糜性肺充血。口服西罗莫司治疗后，她的症状和肺部浸润得到改善；此外，血清肺肿瘤标志物-6（KL-6）水平与这些并发症的症状和肺部浸润情况平行。我们认为血清KL-6可能是LAM中乳糜痰和乳糜性肺充血的有用监测生物标志物。
