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Frontiers of Medicine >> 2013, Volume 7, Issue 1 doi: 10.1007/s11684-013-0238-6

A case of thymic Langerhans cell histiocytosis with diabetes insipidus as the first presentation

1. Department of Endocrinology;

2. Department of Thoracic Surgery; 3. Department of Radiology, First Affiliated Hospital of Guangzhou Medical College, Guangzhou 510120, China

Available online: 2013-03-05

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Abstract

Langerhans cell histiocytosis (LCH) is an idiopathic group of reactive proliferative diseases linked to aberrant immunity, pathologically characterized by clonal proliferation of Langerhans cells. LCH rarely involves the thymus. We report a case of thymic LCH with diabetes insipidus as the first presentation, without evidence of myasthenia gravis and without evidenced involvement of the skin, liver, spleen, bones, lungs and superficial lymph nodes. This present case may have important clinical implications. In screening for LCH lesions, attention should be attached to rarely involved sites in addition to commonly involved organs. Follow-up and imageological examination are very important to a final diagnosis.

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