Diffuse Lung Disease: Diffuse Lung Disease |

Lymphangioleiomyomatosis: How Can This Be Dealt With? FREE TO VIEW

Ramona Elena Nedelcu, PhD; Ruxandra Ulmeanu, PhD; Dan Ioan Ulmeanu, PhD; Florin Mihaltan, PhD; Maria Roxana Nemes, PhD; Paraschiva Postolache, PhD
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“Marius Nasta” Institute of Pneumology, Bucharest, Romania

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A199. doi:10.1016/j.chest.2016.02.206
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SESSION TITLE: Diffuse Lung Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Lymphangioleiomyomatosis (LAM), a rare progressive lung disease which almost exclusively affects women of childbearing age, is characterized by cystic remodeling of the lung parenchyma due to proliferation of abnormal smooth muscle cells. As with many other rare diseases, one of the major issues is the lack of awareness amongst healthcare professionals, resulting in a delay from the time of presentation to diagnosis and treatment.

CASE PRESENTATION: The clinical case of a 34-year-old female, smoker (16PY), diagnosed with diffuse alveolar hemorrhage syndrome of unknown etiology in 2013. One year later, she came to the emergency unit due to another episode of hemoptysis after which multiple interdisciplinary checkups were done (cardiology - for embolisation, pneumology, rheumatology that included immunoassay, alpha 1 antitrypsin deficiency and capillaroscopy tests). A new CT scan shown multiple thin walled cysts and focal areas of ground glass opacities. Moreover, the symptoms and lung function tests (DLCO=39.7%; SaO2=70%) were slowly progressive. To excluding other pathologies, biopsy sampling by left video-assisted thoracoscopic surgery (VATS) was performed.

DISCUSSION:Macroscopically: multiple emphysema bubbles, greatest of 5mm diameter. Microscopically: cysts and a multifocal nodular proliferation of immature smooth muscle and perivascular epithelioid cells (LAM cells). Immunohistochemical tests supported the same diagnosis of LAM (HMB45 and ACT - positive). Two months ago the patient suffered an abdominal emergency surgery to extirpate a pelvic mass that compress the bladder. The lesion extended from the retroperitoneum down to the pelvis along the common iliac vessels and caused severe bleeding intra-operative, an life-threatening complication. Histopathological appearance suggests an angiomyolipoma. After surgery she needed for long-term oxygen therapy and made pulmonary rehabilitation sessions becoming a candidate for lung transplantation.

CONCLUSIONS: No effective treatment for lymphangioleiomyomatosis has been found. Due to the rarity of LAM, the diagnosis is often missed initially and delayed. The case presented is uncommon due to its clinical expression, with alveolar hemorrhage syndrome from its onset, unlike the majority of the published cases. Lung transplantation is mandatory for patients with severe pulmonary involvement, but the angiomyolipomas in other locations represent a contraindication.

Reference #1: McCormack FX, FX (2008). “Lymphangioleiomyomatosis: a clinical update”. Chest133:507-516.

Reference #2: Johnson, SR; Cordier, JF; Lazor, R; Cottin, V; Costabel, U; Harari, S; Reynaud-Gaubert, M; Boehler, A; Brauner, M; Popper, H; Bonetti, F; Kingswood, C (2010). “European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis”. Eur Respir J35: 14-26.

Reference #3: Torre, O; Harari, S (2010). “The diagnosis of cystic lung diseases: a role for bronchoalveolar lavage and transbronchial biopsy?”. Respir Med104 (Suppl 1): S81-85.

DISCLOSURE: The following authors have nothing to disclose: Ramona Elena Nedelcu, Ruxandra Ulmeanu, Dan Ioan Ulmeanu, Florin Mihaltan, Maria Roxana Nemes, Paraschiva Postolache

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