Lymphangioleiomyomatosis: An Analysis of Cases Reported In Chinese Literature from 1981 to 2009_Chinese Journal of Respiratory and Critical Care Medicine

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Department of Respiratory Medicine, Anhui Province Hospital. Anhui, Hefei,230001, ChinaCorresponding Author: XU Kai-Feng, E-mail: kaifeng. xu@ gmail. com;

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Keywords：

Lymphangioleiomyomatosis; Diagnosis

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Objective To summarize the clinical features of lymphangioleiomyomatosis ( LAM) in Chinese population. Methods The first case of LAM in China was reported in 1981. All cases of LAM reported in Chinese literature from January 1981 to December 2009 were reviewed. The cases for analysis were included with a diagnosis of LAM confirmed or probable according to the guidelines of European Respiratory Society, and with individual clinical data available. Results A total of 111 cases of LAM were
pooled for analysis. All cases were female. The most frequent onset symptomof LAMwas dyspnea ( 51. 4% ) .The main clinical manifestations included dyspnea ( 93. 4% ) , pneumothorax ( 48. 6% ) , cough ( 45. 7% ) ,hemoptysis ( 35. 1% ) , and chylothorax ( 33. 3% ) . 11 cases were associated with tuberous sclerosis complex.
An obstructive with or without restrictive abnormality in pulmonary function testing was observed in 60 /67 cases. Diffusion impairment was also common ( 45 /46) . Of the 109 patients, computerized tomography of chest showed thin-walled air-filled cysts throughout both lungs in 104 cases, multiple bullae in 3 cases, and
honeycomb changes in 2 cases. 102 cases were diagnosed based on pathological findings. Misdiagnosis was found in 44 cases, the median time of misdiagnosis was 24 months. Conclusion Awareness of LAMshould be raised when an adult female who presents with an unexplained dyspnea, especially combined with repeated
pneumothorax or chylothorax.

Citation： HU Xiaowen,ZHU Jianrong,XU KaiFeng. Lymphangioleiomyomatosis: An Analysis of Cases Reported In Chinese Literature from 1981 to 2009. Chinese Journal of Respiratory and Critical Care Medicine, 2010, 9(5): 508-511. doi: Copy

1.	Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. AmJ Respir Crit Care Med, 2006, 173 : 105-111.
2.	Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J, 2010, 5 : 14 -26.
3.	董瀚基, 冯家昌, 蔡榕, 等. 淋巴管平滑肌瘤病( 结节硬化病的一型) . 中华肿瘤杂志, 1981, 3: 158 .
4.	Xu F, Yang Y, Xia JY, et al. Clinical profiles of pulmonary lymphangioleiomyomatosis in the mainland of China. Chin Med J ( Engl) , 2009, 122: 1473-1476.
5.	卢韶华, 谭云山, 许建芳, 等. 肺淋巴管肌瘤病的临床及病理特点. 中华结核和呼吸杂志, 2009, 9 : 664 -669.
6.	Hayashida M, Seyama K, Inoue Y, et al. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology,2007, 12: 523-530 .
7.	Taveira-DaSilva AM, Hedin C, Stylianou MP, et al. Reversible airflow obstruction, proliferation of abnormal smooth muscle cells,and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis. Am J Respir Crit Care Med, 2001 , 164 :1072-1076.
8.	Bissler JJ, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.N Engl J Med, 2008 , 358: 200-203.
9.	Reynaud-Gaubert M, Mornex JF, Mal H, et al. Lung transplantation for lymphangioleiomyomatosis: the French experience. Transplantation,2008 , 86: 515 -520.
10.	Johnson SR, Whale CI, Hubbard RB, et al. Survival and disease progression in UK patients with lymphangioleiomyomatosis. Thorax,2004, 59: 800-803 .

1. Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. AmJ Respir Crit Care Med, 2006, 173 : 105-111.
2. Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J, 2010, 5 : 14 -26.
3. 董瀚基, 冯家昌, 蔡榕, 等. 淋巴管平滑肌瘤病( 结节硬化病的一型) . 中华肿瘤杂志, 1981, 3: 158 .
4. Xu F, Yang Y, Xia JY, et al. Clinical profiles of pulmonary lymphangioleiomyomatosis in the mainland of China. Chin Med J ( Engl) , 2009, 122: 1473-1476.
5. 卢韶华, 谭云山, 许建芳, 等. 肺淋巴管肌瘤病的临床及病理特点. 中华结核和呼吸杂志, 2009, 9 : 664 -669.
6. Hayashida M, Seyama K, Inoue Y, et al. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology,2007, 12: 523-530 .
7. Taveira-DaSilva AM, Hedin C, Stylianou MP, et al. Reversible airflow obstruction, proliferation of abnormal smooth muscle cells,and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis. Am J Respir Crit Care Med, 2001 , 164 :1072-1076.
8. Bissler JJ, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.N Engl J Med, 2008 , 358: 200-203.
9. Reynaud-Gaubert M, Mornex JF, Mal H, et al. Lung transplantation for lymphangioleiomyomatosis: the French experience. Transplantation,2008 , 86: 515 -520.
10. Johnson SR, Whale CI, Hubbard RB, et al. Survival and disease progression in UK patients with lymphangioleiomyomatosis. Thorax,2004, 59: 800-803 .

Chinese Journal of Respiratory and Critical Care Medicine

Lymphangioleiomyomatosis: An Analysis of Cases Reported In Chinese Literature from 1981 to 2009

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