Chest
Original ResearchLymphangioleiomyomatosisSerum Vascular Endothelial Growth Factor-D Levels in Patients With Lymphangioleiomyomatosis Reflect Lymphatic Involvement
Section snippets
Participants
The research was approved by the Institutional Review Board of the National Heart, Lung, and Blood Institute (protocols 95-H-0186 and 96-H-0100); all participants gave written informed consent. The diagnosis of LAM was made by tissue biopsy and/or clinical and roentgenographic data. The presence or absence of lymphangioleiomyoma and adenopathy in 111 patients with LAM was determined by screening CT scan (model 9800 and Lightspeed scanners; General Electric; Milwaukee, WI) and/or sonography
Results
The study group was composed of 111 patients with sporadic LAM (all women; mean age, 51.4 years) and 40 healthy volunteers (all women; mean age, 49.6 years) [Table 1]. The diagnosis of LAM was confirmed by biopsy or by roentgenographic evidence of characteristic cystic lung lesions and extrapulmonary manifestations (eg, AMLs lymphangioleiomyomas). Subjects presenting with pulmonary involvement alone can be considered as possible false-positive findings. Of the 111 patients in the LAM cohort,
Discussion
A patient with cystic lung disease is likely to have LAM if presenting with extrapulmonary manifestations (eg, AML lymphangioleiomyoma). It is in patients whose disease is restricted to the lung that the diagnostic dilemma occurs. Similar thin-walled cysts may be seen in other diseases characterized by lung involvement. In these cases, an open-lung biopsy is often recommended to confirm LAM. The discovery of a biomarker for diagnosis might obviate the need for an invasive biopsy procedure.
Acknowledgment
We thank Dr. Martha Vaughan, Dr. Wendy Steagall, Dr. Souheil El-Chemaly, and Dr. Gustavo Pacheco-Rodriguez (Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health) for helpful discussions and critical review of the manuscript.
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2020, Medical HypothesesCitation Excerpt :The lymphangiogenic factor VEGF-D is produced by normal mesothelial cells, and VEGF-D expression is sensitive to inhibition by rapamycin in these cells [46]. VEGF-D is elevated in the serum of women with LAM, is of value as a diagnostic biomarker of LAM [47–50] and is strongly associated with lymphatic involvement in LAM [48,51–53]. This is consistent with expansion of a VEGF-D-expressing hyperplastic mesothelial cell population and with elevation of VEGF-D expression in these cells consequent to mTORC activation.
This research was funded by the Intramural Research Program, National Institutes of Health, National Heart, Lung, and Blood Institute.
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).