A Case of Langerhans Cell Histiocytosis with Central Diabetes Insipidus, Hypopituitarism and Pulmonary Involvement

作者：Demirtas Selma^*; Demir Oezguer; Erdal Harun; Erdogan Murat Faik; Beksac Meral

来源：Turkiye Klinikleri Tip Bilimleri Dergisi, 2010, 30(1): 368-374.

DOI：10.5336/medsci.2008-8695

• Diabetes insipidus
• hypopituitarism
• histiocytosis
• Langerhans-cell

Langerhans cell histiocytosis (LCH) is an uncommon, heterogeneous disease group, characterised by the infiltration of abnormal Langerhans cells in various organs. Usually, skin, bone, lymph nodes are affected; involvement of crucial organs like liver, spleen, lungs, bone marrow, worsens the outcome. At present, there is not a universally accepted, standard treatment modality for adult LCH. Here, a 30-year-old patient with LCH in who presented with central diabetes insipidus, hypopituitarism and pulmonary involvement and showed partial response to therapy is reported. The patient was administered to the chemotherapy protocol of the international clinical LCH III study, for the patients with crucial organ involvement, which contains vinblastine, methotrexate, prednisone and 6-mercaptopurine, between March 12, 2007 and February 25, 2008. The response evaluation in the second month after the end of the treatment revealed partial regression. Follow-up with the desmopressin, levo-thyroxine, dexamethasone and testosterone replacement therapy and control for every six months were recommended.

• 出版日期2010-2