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Endocrine Abstracts (2015) 37 EP1154 | DOI: 10.1530/endoabs.37.EP1154

1Serviço de Endocrinologia, Diabetes e Nutrição do Centro Hospitalar do Baixo Vouga, Aveiro, Portugal; 2Serviço de Endocrinologia, Diabetes e Metabolismo do Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Introduction: Langerhan Cells Histiocytosis (LCH) is a rare heterogeneous idiopathic clinical entity involving clonal proliferation of Langerhan cells that may infiltrate most commonly bone, skin, lymph nodes or lungs. It affects mainly children between 5 and 15 years. The most common endocrine manifestation is diabetes insipida.

Clinical case: The authors describe a case of a 19-year-old female patient, admitted in the Transition Follow-up of the Endocrinology Department of Coimbra’s Hospital and Universitary Center. In her past medical history, at the age of 15 months she presented with rash, fever and distended abdomen. Complementary diagnosis exams showed anemia and hepatosplenomegaly and the first bone marrow examination reached the diagnosis of Kala-Azar but the directed treatment did not show clinical improvement. A new bone marrow exam was conducted with a final diagnosis of LCH. After 17 years over the diagnosis, two recurrences and 27 chemotherapy courses she was now referred to Transition Follow-up. On admission she showed no sign of recurrent disease, no anterior-pituitary disfunction, the maintenance of pituitary stalk thickening at MRI and diabetes insipida, treated with desmopressin.

Discussion: Since it affects mostly children, the majority of published literature about HCL concerns pediatric patients. Our patient reached adulthood and now we face the challenge of planning the future follow-up, concerning the risks of recurrence of the primary disease and the consequences and long-term effects of the treatment of a childhood cancer.

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