Background: Langerhans cells histiocytosis (LCH) is due to clonal proliferation of histiocytes and consequently tissue infiltration. LCH has a wide range of clinical manifestations from hypothalamic dysfunction diabetes insipidus to lymph nodes and spleen enlargement, pancytopenia, lung disease and skin involvement. The clinical presentation and course varies from indolent forms or spontaneous remissions to progressive disease and unfortunately refractory to current therapeutic approach. The diagnosis of LCH is based on the histological evaluation of lesional tissue and the demonstration of Birbëck’granules in the cytoplasms of the Langerhans cells by electronic microscope or its characteristic morphology and the lesional histiocytes must be proven to have the specific phenotype (CD1a+, S-100+, LC granules+). The therapy includes different optional agents from esteroids to chemotherapy combinations or radiotherapy. Patients: We describe the last 6 cases diagnosed at the Haematology Department in our Hospital from May 2001 to September 2003.

Case1: A 20-year-old female, with a tumour located in the cranial base diagnosed of LCH in 1999. In May 2001: lung LCH is found by biopsy with hypothalamic dysfunction, pituitary failure and diabetes insipidus. Treatment: Telecobaltotherapy in CNS and chemotherapy (DAL HX-83/90). Reevaluation: CNS MRI normal, body computed tomography (CT) scan residual lung disease. Partial remission, asymptomatic, under hormonal substitutive treatment (HST). In June 2004 diagnosed Myelodendritic Leukemia. A squeduled therapy with 2-chloro-deoxiadenosine has been started.

Case 2: A 20-year-old male treated because of diabetes insipidus since 1997. In november 2001, after several pneumothorax, is diagnosed, by biopsy of lung, LCH with lung disease and hypothalamic-pituitary involvement. Treatment: Chemotherapy (DAL HX-83/90). Reevaluation: CT scan residual lung disease. Partial remission, clinically stable under HST. Case 3: A 21-year-old female. March 2002 after several pneumothorax is diagnosed, by biopsy of lung, LCH with lung disease. Treatment: Chemotherapy (DAL HX-83/90). A severe pneumothorax appeared and led to stop chemotherapy. DI was diagnosed in 2003. Clinically stable under HST. Case 4: A 33-year-old female. Eosinophilic granuloma located in a rib was erradicated by surgery in 1990. In June 2002, she was diagnosed of costal and lung LCH by biopsy. Therapy with cladribine (

Blood 1999; vol 93 (12): 4125–30
). Reevaluation: residual lung disease. Partial remission, clinically stable. Case 5: A 44-year-old female. DI. Diagnosis in May 2003, by biopsy of perianal area, LCH perianal, lung and pituitary. Treatment with cladribine. Reevaluation: Partial remission in skin and lung disease, clynically stable under HST. Case 6:A 20-year-old male. Diagnosis in September 2003 of LCH in bone: S1 vertebra and mass pre-sacred. He was treated with surgery and local radiotherapy. Reevaluation: complete remission without clinical manifestations.

Conclusions: Due to the low incidence or this disease, the number of cases seen during these months has been surprisingly high. The treatment of LCH currently is mainly paliative without a standard treatment. Many different treatments have been tried: corticoesteroids, alquilants agents, antimetabolic agents, vinca alkaloids, different combinations of these agents, radiotherapy and inmunotherapy. Recently new treatments are being included with the aim of improving the results, as 2-chloro-deoxiadenosine. This study has been supported by a grant from FEHHA

Topics:
histiocytosis, langerhans cell, lung diseases, chemotherapy regimen, partial response, biopsy, diabetes insipidus, pneumothorax, radiation therapy, cladribine

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2005, The American Society of Hematology
2004
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