Sporadic lymphangioleiomyomatosis with bloody sputum as an initial symptom: A case report and review of the literature

Wu,Kai; Lu,Hui‑Yu

doi:10.3892/etm.2015.2397

Sporadic lymphangioleiomyomatosis with bloody sputum as an initial symptom: A case report and review of the literature

Authors:
- Kai Wu
- Hui‑Yu Lu
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Affiliations: Department of Respiratory Medicine, Taizhou People's Hospital, Taizhou, Jiangsu 225300, P.R. China
Published online on: March 31, 2015 https://doi.org/10.3892/etm.2015.2397
Pages: 2159-2164

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Abstract

Lymphangioleiomyomatosis (LAM) is an extremely rare lung disease affecting females of a childbearing age. The condition occurs sporadically or in association with tuberous sclerosis complex. The diagnosis of LAM is often delayed since the clinical symptoms and signs are non‑specific. The present study reports the a case of a patient with LAM, in which bloody sputum was presented as the initial symptom. The 43‑year‑old female had experienced a small amount of bloody sputum over the previous five years. The patient was admitted to the Department of Respiratory Medicine at Taizhou People's Hospital (Taizhou, China) in 2012, reporting symptoms of a cough, shortness of breath and bloody sputum over the previous 10 days. A high‑resolution computed tomography scan revealed multiple small circular thin‑walled translucent areas in both lung fields. The initial diagnosis of the patient was LAM. A biopsy was performed using a video‑assisted thoracoscopy. In conclusion, increased awareness and early diagnosis and treatment were determined to be key factors in ensuring a satisfactory prognosis.

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1	Johnson SR, Cordier JF, Lazor R, et al: Review Panel of the ERS LAM Task Force: European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 35:14–26. 2010. View Article : Google Scholar : PubMed/NCBI
2	Meraj R, Wikenheiser-Brokamp KA, Young LR and McCormack FX: Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 33:486–497. 2012. View Article : Google Scholar : PubMed/NCBI
3	Pallisa E, Sanz P, Roman A, et al: Lymphangioleiomyomatosis: pulmonary and abdominal findings with pathologic correlation. Radiographics. 22:S185–S198. 2002. View Article : Google Scholar : PubMed/NCBI
4	Johnson S: Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 54:254–264. 1999. View Article : Google Scholar : PubMed/NCBI
5	Mavroudi M, Zarogoulidis P, Katsikogiannis N, et al: Lymphangioleiomyomatosis: current and future. J Thorac Dis. 5:74–79. 2013.PubMed/NCBI
6	Hancock E and Osborne J: Lymphangioleiomyomatosis: a review of the literature. Respir Med. 96:1–6. 2002. View Article : Google Scholar : PubMed/NCBI
7	Chu SC, Horiba K, Usuki J, et al: Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest. 115:1041–1052. 1999. View Article : Google Scholar : PubMed/NCBI
8	Johnson SR: Lymphangioleiomyomatosis. Eur Respir J. 27:1056–1065. 2006.PubMed/NCBI
9	Ansótegui Barrera E, Mancheño Franch N, Vera-Sempere F and Padilla Alarcón J: Lymphangioleiomyomatosis. Arch Bronconeumol. 47:85–93. 2011.[(In English and Spanish)]. View Article : Google Scholar : PubMed/NCBI
10	Almoosa KF, Ryu JH, Mendez J, et al: Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest. 129:1274–1281. 2006. View Article : Google Scholar : PubMed/NCBI
11	Johnson SR and Tattersfield AE: Clinical experience of lymphangioleiomyomatosis in the UK. Thorax. 55:1052–1057. 2000. View Article : Google Scholar : PubMed/NCBI
12	Ryu JH, Doerr CH, Fisher SD, et al: Chylothorax in lymphangioleiomyomatosis. Chest. 123:623–627. 2003. View Article : Google Scholar : PubMed/NCBI
13	Juvet SC, McCormack FX, Kwiatkowski DJ, et al: Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans. Am J Respir Cell Mol Biol. 36:398–408. 2007. View Article : Google Scholar : PubMed/NCBI
14	Koyama M, Johkoh T, Honda O, et al: Chronic cystic lung disease: diagnostic accuracy of high-resolution CT in 92 patients. AJR Am J Roentgenol. 180:827–835. 2003. View Article : Google Scholar : PubMed/NCBI
15	Taveira-DaSilva AM, Stylianou MP, Hedin CJ, et al: Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone. Chest. 126:1867–1874. 2004. View Article : Google Scholar : PubMed/NCBI
16	Johnson SR and Tattersfield AE: Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment. Am J Respir Crit Care Med. 160:628–633. 1999. View Article : Google Scholar : PubMed/NCBI
17	Avila NA, Chen CC, Chu SC, et al: Pulmonary lymphangioleiomyomatosis: correlation of ventilation-perfusion scintigraphy, chest radiography, and CT with pulmonary function tests. Radiology. 214:441–446. 2000. View Article : Google Scholar : PubMed/NCBI
18	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. 164:1072–1076. 2001. View Article : Google Scholar : PubMed/NCBI
19	Krymskaya VP: Smooth muscle-like cells in pulmonary lymphangioleiomyomatosis. Proc Am Thorac Soc. 5:119–126. 2008. View Article : Google Scholar : PubMed/NCBI
20	Yu J, Astrinidis A, Howard S, et al: Estradiol and tamoxifen stimulate LAM-associated angiomyolipoma cell growth and activate both genomic and nongenomic signaling pathways. Am J Physiol Lung Cell Mol Physiol. 286:L694–L700. 2004. View Article : Google Scholar : PubMed/NCBI
21	Gao J, Zhu P, Zhang S, et al: A clinicopathological analysis of pulmonary lymphangioleiomyomatosis. Zhongguo Fei Ai Za Zhi. 14:378–382. 2011.[(In Chinese)]. PubMed/NCBI
22	Bonetti F, Chiodera PL, Pea M, et al: Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis. Am J Surg Pathol. 17:1092–1102. 1993. View Article : Google Scholar : PubMed/NCBI
23	Meraj R, Wikenheiser-Brokamp KA, Young LR, et al: Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis. Front Med. 4:395–405. 2012. View Article : Google Scholar
24	Glassberg MK, Elliot SJ, Fritz J, et al: Activation of the estrogen receptor contributes to the progression of pulmonary lymphangioleiomyomatosis via matrix metalloproteinase-induced cell invasiveness. J Clin Endocrinol Metab. 93:1625–1633. 2008. View Article : Google Scholar : PubMed/NCBI
25	Riojas RA, Bahr BA, Thomas DB, et al: A case report of lymphangioleiomyomatosis presenting as spontaneous pneumothorax. Mil Med. 177:477–480. 2012. View Article : Google Scholar : PubMed/NCBI
26	Matsui K, Beasley MB, Nelson WK, et al: Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score. Am J Surg Pathol. 25:479–484. 2001. View Article : Google Scholar : PubMed/NCBI
27	Harari S, Torre O and Moss J: Lymphangioleiomyomatosis: what do we know and what are we looking for? Eur Respir Rev. 20:34–44. 2011. View Article : Google Scholar : PubMed/NCBI
28	Yano S: Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment. Thorax. 57:1085–1086. 2002. View Article : Google Scholar : PubMed/NCBI
29	Johnson SR and Tattersfield AE: Clinical experience of lymphangioleiomyomatosis in the UK. Thorax. 55:1052–1057. 2000. View Article : Google Scholar : PubMed/NCBI
30	Fujimoto M, Ohara N, Sasaki H, et al: Pregnancy complicatedwith pulmonary lymphangioleiomyomatosis: case report. Clin Exp Obstet Gynecol. 32:199–200. 2005.PubMed/NCBI
31	Brunelli A, Catalini G and Fianchini A: Pregnancy exacerbating unsuspected mediastinal lymphangioleiomyomatosis and chylothorax. Int J Gynaecol Obstet. 52:289–290. 1996. View Article : Google Scholar : PubMed/NCBI
32	McLoughlin L, Thomas G and Hasan K: Pregnancy and lymphangioleiomyomatosis: anaesthetic management. Int J Obstet Anesth. 12:40–44. 2003. View Article : Google Scholar : PubMed/NCBI
33	Casanova A and Ancochea J: Lymphangioleiomyomatosis: new therapeutic approaches. Arch Bronconeumol. 47:579–580. 2011.[(In Spanish)]. View Article : Google Scholar : PubMed/NCBI
34	Bissler JJ, McCormack FX, Young LR, et al: Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomotosis. N Engl J Med. 358:140–151. 2008. View Article : Google Scholar : PubMed/NCBI
35	Casanova A, María Girón R, Acosta O, Barrón M, Valenzuela C and Ancochea J: Lymphangioleiomyomatosis treatment with sirolimus. Arch Bronconeumol. 47:470–472. 2011.[(In Spanish)]. View Article : Google Scholar : PubMed/NCBI
36	McCormack FX, Inoue Y, Moss J, et al: National Institutes of Health Rare Lung Diseases Consortium; MILES Trial Group: Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 364:1595–1606. 2011. View Article : Google Scholar : PubMed/NCBI
37	Kinder B and McCormack FX: Clinical trials for rare lung diseases; lessons from lymphangioleiomyomatosis. Lymphat Res Biol. 8:71–79. 2010. View Article : Google Scholar : PubMed/NCBI