Lymphangioleiomyomatosis

Lymphangiomyomatosis (LAM) has been reported to recur after lung transplantation, but it is unclear whether the recurrence arises from the patient or from the donor. Karbowniczek and coworkers studied a 44-year-old woman who died of Aspergillus pneumonia at 22 months after lung transplantation for LAM. Specimens obtained from the transplanted lung (allograft) at autopsy were analyzed by microsatellite marker fingerprinting, which revealed patient-derived LAM cells. Before transplantation, a somatic one base pair deletion in exon 18 of the TSC2 gene was identified in LAM cells (lung and lymph nodes). The same mutation was found in the recurrence of LAM, indicating that the recurrence originated in the patient. The authors conclude that histologically benign LAM cells can migrate or metastasize to lung allografts after transplantation.

To determine the causes of exercise intolerance in patients with LAM, Taveira-DaSilva and coworkers undertook cardiopulmonary exercise testing in 217 patients with LAM. Maximum oxygen uptake was decreased in 162 patients, of whom 28 did not reach anaerobic threshold, 29 patients had a low oxygen uptake at anaerobic threshold, and 54 developed hypoxemia. Maximum oxygen uptake decreased as the score for histologic severity increased. Significant predictors of maximum oxygen uptake were FEV₁ and diffusing capacity for carbon monoxide; uptake was also correlated with computed tomography, use of oxygen, and resting PO₂. Some patients with near-normal FEV₁ and diffusing capacity developed hypoxemia during exercise. The authors conclude that cardiopulmonary exercise testing should be performed in patients with LAM to determine the severity of abnormal gas exchange and the need for supplemental oxygen therapy. An editorial commentary by Sietsema and McCormack accompanies this article.

Citations 1-3 of 3 total displayed.

Lymphangioleiomyomatosis: Insights about, and from, a Rare Disease

Kathy E. Sietsema and Francis X. McCormack
Am. J. Respir. Crit. Care Med. 168: 1405-1406. [Full text]

Maximal Oxygen Uptake and Severity of Disease in Lymphangioleiomyomatosis

Angelo M. Taveira-DaSilva, Mario P. Stylianou, Carolyn J. Hedin, Arnold S. Kristof, Nilo A. Avila, Antoinette Rabel, William D. Travis, and Joel Moss
Am. J. Respir. Crit. Care Med. 168: 1427 -1431. First published online as doi:10.1164/rccm.200206-593OC [Abstract] [Full text]

Recurrent Lymphangiomyomatosis after Transplantation: Genetic Analyses Reveal a Metastatic Mechanism

Magdalena Karbowniczek, Aristotelis Astrinidis, Binaifer R. Balsara, Joseph R. Testa, James H. Lium, Thomas V. Colby, Francis X. McCormack, and Elizabeth Petri Henske
Am. J. Respir. Crit. Care Med. 167: 976 -982. First published online as doi:10.1164/rccm.200208-969OC [Abstract] [Full text]

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