LYMPHANGIOLEІOMYOMATOSIS. DIAGNOSIS AND TREATMENT
DOI:
https://doi.org/10.11603/24116-4944.2018.2.8944Keywords:
pneumothorax, recurrent pneumothorax, lymphangioleіomyomatosis (LAM)Abstract
The aim of the study – to learn the features of diagnosis and treatment of patients with pulmonary form of lymphangioleіomyomatosis (LAM).
Materials and Methods. A 26 year-old patient was on treatment at Kyiv City Hospital No. 17 from 07.04.2007 to 16.03.2012. She underwent a complete clinical and diagnostic system of examinations and treatment in accordance with the protocols in force.
Results and Discussion. The article presents a review of the literature on LAM and a description of the clinical case, as well as the features of diagnosis and treatment of LAM.
Modern methods of examination, surgical interventions and rational conservative therapy are the method of choice in diagnosing and treating LAM. Comprehensive examination is a prerequisite for choosing a rational tactic for further treatment.
Conclusions. Until now, LAM remains an undiagnosed disease, and its prevalence in the world population reaches 100.000. The optimal treatment results are achieved with a properly established diagnosis.
References
von Stossel, E. (1937). Uber muskulare Cirrhose der Lunge (Muscular cirrhosis of the lung). Beitr. Klin. Tuberk., 90, 432-442.
Harknett, E.C., Chang, W.Y.C., Byrnes, S., Johnson, J., Lazor, R., Cohen, M.M., B. Gray, … Johnson, S.R. (2011). Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis. QJM., 104 (11), 971-979.
Kitaichi, M., Nishimura, K., Itoh, H., & Izumi, T. (1995). Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors. Am. J. Respir. Crit. Care Med., 151 (2 Pt 1), 527-533.
McCormack, F.X. (2008). Lymphangioleiomyomatosis: a clinical update. Chest, 133 (2), 507-516.
Johnson, S.R. (2006). Lymphangioleiomyomatosis. Eur. Respir. J., 27 (5), 1056-1065.
Taveira-DaSilva, A.M., Steagall, W.K., & Moss, J. (2006). Lymphangioleiomyomatosis. Cancer Control., 13 (4), 276-285.
Miyake, M., Tateishi, U., Maeda, T., Kusumoto, M., Sata-ke, M., & Arai, Y. (2005). Pulmonary lymphangioleiomyomatosis in a male patient with tuberous sclerosis complex. Radiat. Med., 23 (7), 525-527.
Kang, H.W., Kim, C.J., Kang, S.K., Lee, K.S., Lee, C.S., & Kim, Y.H. (1991). Pulmonary lymphangioleiomyomatosis in a male. J. Korean. Med. Sci., 6 (1), 83-85.
Franz, D.N., Brody, A., Meyer, C., Leonard, J., Chuck, G., Dabora, S., … Colby, T.V. (2001). Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am. J. Respir. Crit. Care Med., 164 (4), 661-668.
Costello, L.C., Hartman, T.E., & Ryu, J.H. (2000). High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. Mayo Clin. Proc., 75 (6), 591-594.
Moss, J, Avila, N.A., Barnes, P.M., Litzenberger, R.A., Bechtle, J., Brooks, P.G., & Hedin, C.J. (2001). Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am. J. Respir. Crit. Care Med., 164 (4), 669-671.
Maruyama, H., Ohbayashi, C., Hino, O., Tsutsumi, M., Konishi, Y. (2001). Pathogenesis of multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2. Pathol. Int., 51, 585-594.
Henske, E.P., & McCormack, F.X. (2012). Lymphangioleiomyomatosis – a wolf in sheep’s clothing. The Journal of Clinical Investigation, 122 (11). http://www.jci.org
Smolarek, T.A., Wessner, L.L., McCormack, F.X., Mylet, J.C., Menon, A.G., & Henske, E.P. (1998). Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis. Am. J. Hum. Genet, 62, 810-815.
Carsillo, T., Astrinidis, A., & Henske, E.P. (2000). Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. Proc. Natl. Acad. Sci. USA, 97, 6085-6090.
Yu, J., Astrinidis, A., & Henske, E.P. (2001). Chromosome 16 loss of heterozygosity in tuberous sclerosis and sporadic lymphangiomyomatosis. Am. J. Respir. Crit. Care Med., 164, 1537-1540.
Bonetti, F., Chiodera, P.L., Pea, M., & Martignoni, G. (1993). Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis. Am. J. Surg. Pathol., 17, 1092-1102.
Young, L.R., Vandyke, R., Gulleman, P.M., Inoue, Y., Brown, K.K., Schmidt, L.S., … Linehan, W.M. (2010). Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest., 138 (3), 674-681.
Xu, K.F., Zhang, P., Tian, X., Ma, A., Li, X., Zhou, J., … Zeng, N. (2013). The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis (LAM). Respir. Med., 107 (2), 263-268.
Glasgow, C.G., Avila, N.A., Lin, J.P., Stylianou, M.P., & Moss, J. (2009). Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement. Chest., 135 (5), 1293-1300.
Hayashida, M., Seyama, K., Inoue, Y., Fujimoto, K., & Kubo, K. (2007). Respiratory Failure Research Group of the Japanese Ministry of Health, Labor, and Welfare. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology, 12, 523-530.
Steagall, W.K., Taveira-DaSilva, A.M., & Moss, J. (2005). Clinical and molecular insights into lymphangioleiomyomatosis. Sarcoidosis Vasc. Diffuse Lung Dis., 22 (Suppl. 1), S49-66.
Lu, H.C., Wang, J., Tsang, Y.M., Lin, M.C., & Li, Y.W. (2003). Lymphangioleiomyomatosis initially presenting with abdominal pain: a case report. Clin. Imaging, 27 (3), 166-170.
Matsui, K., Tatsuguchi, A., Valencia, J., Yu, Z., Bechtle, J., & Beasley, M.B. (2000). Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum. Pathol., 31 (10), 1242-1248.
Johnson, S.R., Whale, C.I., Hubbard, R.B., Lewis, S.A., & Tattersfield, A.E. (2004). Survival and disease progression in UK patients with lymphangioleiomyomatosis. Thorax, 59 (9), 800-803.
Ryu, J.H., Moss, J., Beck, G.J., Lee, J.C., Brown, K.K., & Chapman, J.T. (2006). The NHLBI Lymphangioleiomyomatosis Registry. Characteristics of 230 Patients at Enrollment. Am. J. Respir. Crit. Care Med., 173, 105-111.
Taveira-DaSilva, A.M., Jones, A.M., Julien-Williams, P., Yao, J., Stylianou, M., Moss, J. (2015). Severity and outcome of cystic lung disease in women with tuberous sclerosis complex. Eur. Respir. J., 45, 171-180.
Taveira-DaSilva, A.M., Stylianou, M.P., Hedin, C.J., Hathaway, O., & Moss, J. (2004). Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone. Chest, 126, 1867-1874.
Almoosa, K.F., Ryu, J.H., Mendez, J., Huggins, J.T., Young, L.R., Sullivan, E.J., … Sahn, S.A. (2006). Management of pneumothorax in lymphangioleiomyomatosis: effect on recurrence and lung transplantation complications. Chest, 129, 1274-1281.
Hohman, D.W., Noghrehkar, D., & Ratnayake, S. (2008). Lymphangioleiomyomatosis: a review. Eur. J. Intern. Med., 19, 319-324.
Taveira-DaSilva, A.M., Burstein, D., Hathaway, O.M., Fontana, J.R., Gochuico, B.R., Avila, N.A., & Moss, J. (2009). Pneumothorax after air travel in lymphangioleiomyomatosis, idiopathic pulmonary fibrosis, and sarcoidosis. Chest, 136, 665-670.
(2002). Managing passengers with respiratory disease planning air travel: British Thoracic Society recommendations. Thorax, 57, 289-304.
Hu, X., Cowl, C.T., Baqir, M., & Ryu, J.H. (2014). Air travel and pneumothorax. Chest, 145, 688-694.
Cohen, M.M., Freyer, A.M., & Johnson, S.R. (2009). Pregnancy experiences among women with lymphangioleiomyomatosis. Respir. Med., 103, 766-772.
Nishant Gupta, Geraldine A. Finlay, Robert M. Kotloff, Charlie Strange, Kevin C. Wilson, Lisa R. Young, et al. AMERICAN THORACIC SOCIETY DOCUMENTS Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline on behalf of the ATS Assembly on Clinical Problems.
Johnson, S.R., Cordier, J.F., Lazor, R., Cottin, V., Costabel, U.S., Harari, M., … Kingswood, C. (2010). European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. European Respiratory Journal, 35, 14-26. DOI: 10.1183/09031936.00076209.
McCormack, F.X., Inoue, Y., Moss, J., Singer, L.G., Strange, C., Nakata, K., … Trapnell, B.C. (2011). Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N. Engl. J. Med., 364, 1595-1606.
Taveira-DaSilva, A.M., Hathaway, O., Stylianou, M., & Moss, J. (2011). Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus. Ann. Intern. Med., 154, 797-805, W-292-3.
Bissler, J.J., McCormack, F.X., Young, L.R., Elwing, J.M., Chuck, G., Leonard, J.M., … Franz, D.N. (2008). Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N. Engl. J. Med., 358, 140-151.
Pechet, T.T., Meyers, B.F., Guthrie, T.J., Battafarano, R.J., Trulock, E.P., Cooper, J.D., & Patterson, G.A. (2004). Lung transplantation for lymphangioleiomyomatosis. J Heart Lung Transplant., 23, 301-308.
Kpodonu, J., Massad, M.G., Chaer, R.A., Caines, A., Evans, A., Snow, N.J., & Geha, A.S. (2005). The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis. J. Heart Lung Transplant., 24, 1247-1253.
Maurer, J.R., Ryu, J., Beck, G., Moss, J., Lee, J., Finlay, G., … Sherer, S. (2007). Lung transplantation in the management of patients with lymphangioleiomyomatosis: baseline data from the NHLBI LAM Registry. J. Heart Lung Transplant., 26, 1293-1299.
Reynaud-Gaubert, M., Mornex, J., Mal, H., Treilhaud, M., Dromer, C., Quétant, S., … Stern, M. (2008). Lung transplantation for lymphangioleiomyomatosis: the French experience. Transplantation, 86, 515-520.
Downloads
Published
Issue
Section
License
Authors who publish in this journal agree to the following terms:
1. The authors reserve the right to authorship of the work and pass the journal right of first publication of this work is licensed under a Creative Commons Attribution License, which allows others to freely distribute the work published with reference to the authors of the original work and the first publication of this magazine.
2. Authors are entitled to enter into a separate agreement on additional non-exclusive distribution of work in the form in which it was published in the magazine (eg work place in the electronic repository institution or publish monographs in part), provided that the reference to the first publication of this magazine.
3. Policy magazine allows and encourages authors placement on the Internet (eg, in storage facilities or on personal websites) manuscript of how to submit the manuscript to the editor and during his editorial processing, since it contributes to productive scientific discussion and positive impact on the efficiency and dynamics of citing published work (see. The Effect of Open Access).
