- 期刊
淋巴血管平滑肌增生症合併反覆氣胸及乳糜胸－病例報告
Lymphangioleiomyomatosis Combining Recurrent Pneumothorax and Chylothorax：A Case Report
摘要
淋巴血管平滑肌增生症(lymphangioleiomyomatosis,LAM)是一種罕見的進行性疾病，好發於生育年齡的婦女。主要是因為肺部產生不正常的平滑肌細胞(atypical smooth muscle-like cells, LAMcells)增生，造成阻塞性肺部疾病，甚至是氣胸、乳糜胸，腹腔腫瘤等。胸部電腦斷層影像裡可發現LAM的典型特徵：廣泛瀰漫性薄壁肺囊腫病變(diffuse thin-walled cysts throughout the pulmonaryparenchyma)，可做為鑑別診斷，但確診仍以病理切片為主。目前尚無有效治療方法，主要還是著重於預防或控制併發症。報告案例為一育齡婦女，因呼吸喘而至急診就醫，胸部X光報告顯示為右側氣胸及肋膜積水，予胸管置入治療並入院治療。胸部電腦斷層報告及臨床症狀高度懷疑為LAM；因反覆氣胸及乳糜胸，予外科手術介入治療，並行肺部活體組織穿刺送檢確診為LAM。個案發病後因病情進展快速，肺功能持續惡化，呼吸衰竭而死亡。由於此疾病較為罕見，故提出來做經驗分享及疾病介紹。
並列摘要
Lymphangioleiomyomatosis (LAM) is a rare progressive disease, Almost occur in women of reproductive age. Mainly because the lungs can cause abnormal smooth muscle cells (LAM cell) hyperplasia, Cause obstructive pulmonary disease, or even pneumothorax, chylothorax, abdominal tumors. Chest CT images can be found in the typical characteristics of LAM: diffuse thin-walled cysts throughout the pulmonary parenchyma, Can be used as differential diagnosis, but the diagnosis or in biopsy-based. There is no effective treatment, mainly focused on the prevention or control of complications. We report a case of women of childbearing age, to emergency department visits due to difficulty breathing; Chest X-ray is reported as a right pneumothorax and pleural effusion, To chest tube placement and treatment of hospital revenue. Chest CT reports and clinical symptoms highly suspected LAM; due to repeated pneumothorax and chylothorax, to surgical intervention treatment, And lung tissue diagnosed with LAM. Case after the onset of lung function continued to deteriorate, difficulty weaning, thus making this case to do the sharing of experiences and disease introduction.
參考文獻
延伸閱讀
- Hsu, I. L., Fong, Y., & Su, C. C. (2004). 縱膈腔囊狀淋巴管瘤併發術後乳糜胸：一則病例報告. Formosan Journal of Surgery, 37(1), 39-42. https://doi.org/10.29833/FJS.200402.0008
- 蔡偉宏、蕭富源、吳佩儒、何上芸、蔡政宏、陳正雄（2011）。淋巴血管平滑肌瘤臨床表現腹腔腫塊合併乳糜腹水－病例報告及文獻回顧。重症醫學雜誌，12(4)，205-211。https://doi.org/10.30035/TCCM.201112.0008
- Lin, C. Y., Wu, C. H., Huang, C. H., & Huang, T. W. (2017). 瀰漫性大型B細胞淋巴癌合併大量胸水病例報告. 胸腔醫學, 32(5), 226-231. https://www.airitilibrary.com/Article/Detail?DocID=10239855-201710-201709270016-201709270016-226-231
- Chen, K. Y., Hsieh, M. J., Lin, M. C., Tsai, Y. H., & Tsao, T. C. (2001). 肺淋巴血管平滑肌增生症以類似支氣管氣喘表現之：病例報告. 胸腔醫學, 16(4), 282-288. https://www.airitilibrary.com/Article/Detail?DocID=10239855-200112-16-4-282-288-a
- Yen, J. H., Tsai, W. C., Ou, T. T., Lin, S. C., Chen, C. J., & Liu, H. W. (2001). Systemic Lupus Erythematosus Complicated by Recurrent Spontaneous Pneumothorax- a Case Report. The Kaohsiung Journal of Medical Sciences, 17(10), 540-544. https://doi.org/10.6452/KJMS.200110.0540