Retrospective analysis of seven patients with adult-onset langerhans cell histiocytosis syndromes: A single center experience.
Langerhans cell histiocytosis syndromes have been described as encompassing a range of disorders, such as eosinophilic granuloma, Letterer-Siwe syndrome and Hand-Schüller-Christian disease. These disorders have been mainly diagnosed at early ages of life and are relatively rare entities in adult age groups. In this study, we aimed to retrospectively evaluate the patients with Langerhans cell histiocytosis followed-up in our hospital. Seven patients were treated between 1995 and 2005. Median age of patients was 27.5 (18-40) years. Main complaints were classified as bone pain in multiple sites (100%), polydipsia (28%), lung infiltration (14%), oral mucosal infiltration (14%), and cranial nerve infiltration (14%). Two patients were diagnosed as Hand-Schüller-Christian disease, and the others were accepted as eosinophilic granuloma. There was no bone marrow or any other organ infiltration except lung infiltration in one patient. Bone infiltration was the prominent sign in all patients with a minimum of one to maximum of seven different sites. All patients were alive during the follow-up period. All patients were treated with radiotherapy except one patient treated with chemotherapy regimen started with vincristine plus dexamethasone and continued with cladribine. Three of seven patients were treated with combined modality, one patient with only chemotherapy and the others with only radiotherapy. There was no grade 3-4 hematological or systemic side effects of treatment. Relapses were detected in only two patients as new bone infiltrations which responded completely to radiotherapy. Langerhans cell histiocytosis syndromes have a relatively benign course in adult patients and can be treated with either radiotherapy or chemo-radiotherapy successfully.
Fahri
F
Şahin
Kezban Pınar
KP
Özen
Filiz
F
Vural
Serra
S
Kamer
Mehmet
M
Argın
Yavuz
Y
Anacak
Mine
M
Hekimgil
Güray
G
Saydam