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Langerhans Cell Histiocytosis
| Content Provider | Scilit |
|---|---|
| Author | Postini, Anna Maria Prever, Adalberto Brach del Pagano, Manuela Rivetti, Elisa Berger, Massimo Asaftei, Sebastian Dorin Barat, Veronica Andreacchio, Antonio Fagioli, Franca |
| Copyright Year | 2012 |
| Description | Journal: Journal of pediatric hematology/oncology 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. |
| Related Links | https://iris.unito.it/bitstream/2318/1690678/2/mph.0b013e318257a6ea.pdf |
| Ending Page | 358 |
| Page Count | 6 |
| Starting Page | 353 |
| ISSN | 10774114 |
| e-ISSN | 15363678 |
| DOI | 10.1097/mph.0b013e318257a6ea |
| Journal | Journal of pediatric hematology/oncology |
| Issue Number | 5 |
| Volume Number | 34 |
| Language | English |
| Publisher | Ovid Technologies (Wolters Kluwer Health) |
| Publisher Date | 2012-07-01 |
| Access Restriction | Open |
| Subject Keyword | Journal: Journal of pediatric hematology/oncology Pediatrics and Child Health Diabetes Insipidus Neurodegeneration |
| Content Type | Text |
| Resource Type | Article |
| Subject | Hematology Pediatrics, Perinatology and Child Health Oncology |
