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Successful Thalidomide Treatment of Persistent Chylous Pleural Effusion

Rachel Pauzner, MD; Haim Mayan, MD; Ana Waizman, MD; Judith Rozenman, MD; and Zvi Farfel, MD
[+] Article and Author Information

From Sheba Medical Center and Tel Aviv University, Tel Hashomer, Israel.


Potential Financial Conflicts of Interest: None disclosed.


Ann Intern Med. 2007;146(1):75-76. doi:10.7326/0003-4819-146-1-200701020-00022
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Grahic Jump Location
Figure.
Course of the patient's disease.

Thoracenteses (solid circles) and an episode of pneumothorax (asterisk) are shown. Drug dosages are stated in the text. The patient was followed until November 2006.

Grahic Jump Location

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lymphangiomatosis and lymphangioleiomyomatosis: phonetically similar but clinically far apart
Posted on June 18, 2007
Eyal Meltzer
the Sheba Medical Center and Tel Aviv University
Conflict of Interest: None Declared

In their recent communication, Pauzner et al. report on a patient with diffuse lymphangiomatosis. one of the treatment options mentioned is anti estrogen therapy. As the authors correctly cite, this treatment is effective in patients with lymphangioleiomyomatosis. However, these phonetically similar conditions are infact totally dissimilar clinically. Diffuse Lymphangiomatosis is a rare condition, that is rarely reported in adults. Is results from a deranged, non neoplastic proliferation of lymphatic vessels which can involve many organs. In fact, the patient described, with her massive skeletal involvement probably represents the subtype of diffuse Lymphangiomatosis that is usually referred to as Gorham Stout disease. Lymphangioleiomyomatosis in a condition that occurs in young women, and is caused by a proliferation of smooth muscle cells in a variety of organs. The usual hallmarks of the disease are lymphadenopathy and parenchymatous lung disease. In this condition anti estrogen therapy can result in a complete cure. This treatment however is not beneficial in diffuse Lymphangiomatosis. The authors are not the first to be misled by the unfortunate similarity in the names of these two diseases. Indeed, a simple MEDLINE search for either of these conditions results in a "mixed bag" of reports of both. Since both diseases can present as chylous effusions, but are totally dissimilar in their treatment and prognosis, clinicians should take care to distinguish between the two.

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