PULMONARY LYMPHANGIOMATOSIS REVEALED BY AN INTERSTITIAL SYNDROME IN A YOUNG ADULT
Keywords:
Lymphangiomatosis, Lung, Thoracic wallAbstract
Background: Diffuse pulmonary lymphangiomatosis is an extremely rare congenital lesion affecting the lymphatic pulmonary system. It is carcterized by a multifocal proliferation and a pulmonary lymphatic vessels dilatation. It is essentially observed in newborns and infants. The diagnosis is based on histological
findings.
Aims: The purpose of this article is to describe the main clinical and pathological features of this rare entity difficult to diagnose.
Case: We report a case of a 19-year old girl who presented with a 2- month standing dyspnea. Radiographic findings consisted in a soft tissue infiltration and thickening in the two lobes. Based on histological and immunohistochemical findings, the diagnosis of diffuse pulmonary lymphangiomatosis was retained.
Conclusion: Diffuse pulmonary lymphangiomatosis is a difficult diagnosis which can be confused with lymphangioleiomyomatosis. Its worse prognosis could be improved by an early diagnosis enabling the use of interferon alpha 2B at the proper time.
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References
Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000 ; 161 : 1037 - 46
Abou Hamdan K, Chabot F, Capron F, Debelle L, Fery M, Polu JM. Diffuse pulmonary lymphangiomatosis in a young adult. Rev Mal Respir 2001 ; 18 : 436 - 9.
El Hajj L, Mazières J, Rouquette I, Mittaine M, Bolduc JP, Didier A et al. Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis : case report and review of the literature. Clin Radiol 2005; 60 : 921 - 5.
Yekeler E, Dursun M, Yildirim A, Tunaci M. Diffuse pulmonary lymphangiomatosis : imaging findings. Diagn Interv Radiol 2005; 11 : 31 - 4
Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC. World Health Organisation Classification of Tumours. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. IARC Press, Lyon 2004 : 104.
Tazelaar HD, Kerr D, Yousem SA, Saldana MJ, Langston C, Colby TV. Diffuse pulomonary lymphangiomatosis. Hum Pathol 1993; 24 : 1313 - 22
Alvarez OA, Kjellin I, Zuppan CW. Thoracic lymphangiomatosis in a child. J Pediatr Hematol Oncol 2004; 26 : 136 - 41
Timke C, Hrause MF, Oppermann HC, Leuschner I, Claviez A. Interferon alpha 2b treatment in an eleven-year-old boy with disseminated lymphangiomatosis. Pediatr Blood Cancer 2007; 48: 108 - 11
