These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


PUBMED FOR HANDHELDS

Search MEDLINE/PubMed


  • Title: Langerhans cell histiocytosis: 40 years' experience.
    Author: Maria Postini A, del Prever AB, Pagano M, Rivetti E, Berger M, Asaftei SD, Barat V, Andreacchio A, Fagioli F.
    Journal: J Pediatr Hematol Oncol; 2012 Jul; 34(5):353-8. PubMed ID: 22627580.
    Abstract:
    OBJECTIVES: Our study analyzes 40 years' experience with pediatric Langerhans cell histiocytosis patients. MATERIALS AND METHODS: Between June 1968 and December 2009, 121 patients (79 males, 42 females; median age 4.13 y) were diagnosed at our center (74% monosystemic disease; 26% multisystemic), treated according to current protocols. We evaluated the response, the survival, and the neuroendocrinological sequelae. RESULTS: Overall survival (OS) for all patients was 93% at 10 years from diagnosis, event-free survival (EFS) 77%. OS for patients younger than 2 years and older than or equal to 2 years was 82% and 97% (P = 0.003); EFS 48% and 87% (P = 0.001). OS for patients diagnosed before and after April 1, 1991 was 84% and 98% (P = 0.007), EFS 66% and 85% (P = 0.03). OS for monosystemic and multisystemic disease was 100% and 71% (P < 0.001); EFS 88% and 45% (P < 0.001). OS for "risk" patients (involvement of bone marrow, spleen, liver, lungs) and "low-risk" patients was 50% and 94% (P = 0.007), EFS 37% and 54% (P = 0.06). Fourteen patients developed diabetes insipidus, 7 patients growth hormone deficiency, 2 hypothyroidism, and 1 neurodegeneration. CONCLUSIONS: Our study confirms improvement of pathogenetic knowledge and treatment over the last 20 years. Age at diagnosis older than or equal to 2 years and standardized treatment are associated with improved prognoses. Multisystemic involvement, especially with "risk" organs seem to be correlated to a worse outcome.
    [Abstract] [Full Text] [Related] [New Search]