Langerhans Cell Histiocytosis
Conditions
Interventions
- Patients in group 1 (single system disease at risk): treatment with Prednisone, Vinblastine and Mercaptopurine.
Initial treatment: Prednisone 1 mg/kg/day ( not to exceed 60 mg) as a 4-week course,
total duration of treatment will be the object of randomization: 6 months vs 12 months.
- Patients in group 3 (isolated pulmonary disease): an observational period of 6 months afer cigarette smoke w
Sponsors
VU University Medical Center Amsterdam,
Department of Hematology
Eligibility
Inclusion criteria
Inclusion criteria: 1. definitive diagnosis of LCH 2. no prior cytoreductive treatment for LCH 3. age 18-50 years for group 1 and 2 4. age 18-75 years for group 3
Exclusion criteria
Exclusion criteria: 1. patients with severe impairment of clinical condition including severely impaired pulmonary function, long term oxygen therapy or cor pulmonale. 2. treatment with immune suppressive agents and/or bisphosphonates within 4 weeks from baseline evaluation <br 3. pregnancy
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Definition and implementation of an uniform treatment for patients with single system LCH, multisystem LCH and pulmonary isolated LCH; implementation of uniform initial evaluation and stratification criteria. | — |
Secondary
| Measure | Time frame |
|---|---|
| - to explore the therapeutic efficacy on adult patients of the standard regimen for multisystem LCH in children, i.e. the combination of vinblastine and prednisone. Endpoints will be survival, reactivation-free survival, permanent sequelae. - to examine if an extended continuation therapy may reduce disease reactivations occuring after treatment completion in patients with multisystem LCH. - to describe the natural history of isolated pulmonary disease and in particular the role of smoking cessation on the disease course. - to explore the therapeutic efficacy of steroid monotherapy in adult patients with isolated pulmonary disease showing disease progression. | — |
Contacts
Public ContactA.A. Loosdrecht, van de
VU University Medical Center Department of Hematology De Boelelaan 1117
Outcome results
None listed