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


Demirtas S., Demir O., Erdal H., ERDOĞAN M. F., BEKSAÇ M.

TURKIYE KLINIKLERI TIP BILIMLERI DERGISI, cilt.30, sa.1, ss.368-374, 2010 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 30 Sayı: 1
  • Basım Tarihi: 2010
  • Doi Numarası: 10.5336/medsci.2008-8695
  • Dergi Adı: TURKIYE KLINIKLERI TIP BILIMLERI DERGISI
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.368-374
  • Anahtar Kelimeler: Diabetes insipidus, hypopituitarism, histiocytosis, Langerhans-cell, FEATURES, CHILDREN
  • Ankara Üniversitesi Adresli: Evet

Özet

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.