A Case of Multisystemic Langerhans Cell Histiocytosis in an Adult

Chng Chiaw Ling

doi:10.15605/jafes.026.01.15

A Case of Multisystemic Langerhans Cell Histiocytosis in an Adult

Authors

Chng Chiaw Ling Singapore General Hospital

DOI:

https://doi.org/10.15605/jafes.026.01.15

Keywords:

multisystemic langerhans cell histiocytosis, lymphangioleiomyomatosis

Abstract

A 23-year-old female smoker presented in late 2008 with a large left pneumothorax. She had multiple spontaneous pneumothoraces in 2007 which required pleurectomy. A presumptive diagnosis of lymphangioleiomyomatosis (LAM) was made in 2007 after the high-resolution computed tomography (HRCT) of the thorax revealed bilateral interlobular septal thickening and multiple thin walled cystic air spaces without lobar predilection, and with preservation of lung volumes.

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References

Nezelof C, Frileux-Herbet F, Cronier-Sachot J. Disseminated histiocytosis X: analysis of prognostic factors based on a retrospective study of 50 cases. Cancer 1979;44:1824-38.

Ryu JH, Moss J, Beck GJ et al. The NHLBI lymphangio-leiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med 2006;173:105-11.

Carsillo T, Astrinidis A, Henske EP. Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. Proc Natl Acad Sci U S A 2000;97:6085-90.

Astrinidis A, Henske EP. Tuberous sclerosis complex: linking growth and energy signaling pathways with human disease. Oncogene 2005;24:7475-81.

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Published

2024-08-23

How to Cite

Ling, C. C. (2024). A Case of Multisystemic Langerhans Cell Histiocytosis in an Adult. Journal of the ASEAN Federation of Endocrine Societies, 26(1), 69. https://doi.org/10.15605/jafes.026.01.15

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Issue

Vol. 26 No. 1 (2011): May Issue

Section

Images in Endocrinology

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