Lymphangioleiomyomatosis: A Case Report

Borovansky, Jill A.; Labonte, Helene R.; Boroff, Erika; Ruddy, Barbara E.; Mayer, Anita P.

doi:10.1089/jwh.2008.0967

Cited by 6 publications

(3 citation statements)

References 22 publications

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“…It causes a proliferation of atypical smooth muscle cells within the lungs, resulting in increasing dyspnea and respiratory failure. 3 This patient was an excellent candidate for transplantation, and after months on the waiting list, she received a deceased single lung transplant. The patient did well after surgery, breathing easily without supplemental oxygen.…”

Section: Case Studymentioning

confidence: 99%

Ethics at the End of Life in Transplant Recipients

Stecher

2011

Prog Transpl

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The field of transplantation is intended to try to save or improve people's lives. In some situations though, the length of a person's life is not as vital as the quality of that life. As a member of a transplant team, one must recognize the differences between quality and quantity and apply the ethical principles of autonomy, justice, nonmaleficence, and beneficence. When a lung transplant recipient became ill with posttransplant lymphoproliferative disease 10 months after her surgery, these ethical principles came to the foreground. This dynamic, vibrant woman, recently engaged, faced her own mortality with grace. There is a time for everything. When death is a long time coming, many people are referred to hospice care and the death is viewed as a blessing. This situation rarely occurs in the transplant field. Many people, whether professionals or laypersons, have a difficult time letting go. Our team and this very special woman all knew when it was time. Creating a warm and open environment in the intensive care unit helped her through the rhythms of transition in dying.

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Section: Case Studymentioning

confidence: 99%

Ethics at the End of Life in Transplant Recipients

Stecher

2011

Prog Transpl

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“…Lymphangiomyomatosis (LAM) is a rare progressive multisystem disease that almost exclusively affects women of childbearing age as either a sporadic form (S-LAM) or as part of a syndrome linked to the autosomal dominant disorder tuberous sclerosis LAM. 1,2 The incidence for S-LAM is 1-2.6 cases per 1,000,000 women. 3 The disorder is characterized by a diffuse proliferation of abnormal smooth muscle cells characteristic of a group of proliferative tumors termed perivascular epithelioid cell tumors.…”

Section: Introductionmentioning

confidence: 99%

Chylous Ascites as a Presentation of Lymphangioleiomyomatosis

et al. 2021

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A 35-year-old woman presented to the hospital with a 4-week history of large-volume chylous ascites refractory to paracentesis and new-onset dyspnea. Thoracic computed tomography revealed diffuse pulmonary cystic lesions with pleural effusions, and abdominal computed tomography showed ascites with large bilateral retroperitoneal masses displaying positron emission tomography avidity. Biopsy of the masses demonstrated lymphatic invasion by a perivascular epithelioid cell neoplasm, a smooth muscle tumor. The patient was diagnosed as having the sporadic form of lymphangioleiomyomatosis and was treated with the mammalian target of rapamycin pathway inhibitor sirolumus with clinical improvement.

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“…Although exceptions have been identified [13][14][15][16][17][18], the vast majority of LAM patients fall into the category of post-pubescent but pre-menopausal women. This observation would strongly suggest estrogen hormone signaling plays a role in LAM pathogenesis, and is further supported by published observations that pregnancy in women with LAM seems to exacerbate the disease while anti-estrogen therapies including oophorectomy [19], ovarian *Address correspondence to this author at the Department of Molecular and Cellular Biochemistry, University of Kentucky, 741 South Limestone Ave., Lexington, KY 40536, USA; Tel: (859) 257-7498; Fax: (859) 323-1037; E-mail: dnoonan@uky.edu irradiation [20] and progesterone administration [9,21] appear to lessen the severity of the disease.…”

Section: Introductionmentioning

confidence: 99%

Evidence for Epigenetic Changes in the Estrogen Receptor Alpha Promoter in Lymphangioleiomyomatosis (LAM)~!2010-01-05~!2010-03-12~!2010-05-11~!

Lou¹

2010

TOLCJ

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Lymphangioleiomyomatosis (LAM) is a rare but often fatal disease, characterized by the abnormal proliferation of smooth muscle cells of the lung. LAM occurs almost exclusively in women and its pathology correlates with mutations in the tuberous sclerosis complex 2 (TSC2) gene and expression of the hormone estrogen. One of the hallmarks of LAM lesions is the anomalous expression of the intracellular receptor for estrogens, ER , and the distinct gender specificity of LAM would support the hypothesis that this anomalous expression of ER plays an essential role in the pathology of the disease. Our previous studies have defined a direct link between the TSC2 gene product tuberin and estrogen signaling through ER . The objectives of this study were to investigate epigenetic changes in the ER promoter as a mechanism for upregulation of ER expression in LAM disease. The results of this study provide evidence for: a) use of multiple ER promoters in human airway smooth muscle cells and LAM-associated cell lines, b) epigenetic changes in the promoter of the ER gene in LAM-associated cell lines and LAM lesions, and c) differential binding of histone deacetylase 1 and methyl-CpG binding proteins in human airway smooth muscle and LAM cells. These studies cumulatively suggest the upregulation of ER expression associated with LAM disease may in part be a consequence of demethylation at the ER promoter.

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Lymphangioleiomyomatosis: A Case Report

Cited by 6 publications

References 22 publications

Ethics at the End of Life in Transplant Recipients

Ethics at the End of Life in Transplant Recipients

Chylous Ascites as a Presentation of Lymphangioleiomyomatosis

Evidence for Epigenetic Changes in the Estrogen Receptor Alpha Promoter in Lymphangioleiomyomatosis (LAM)~!2010-01-05~!2010-03-12~!2010-05-11~!

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