To define and implement an uniform initial evaluation and stratification on adult patients with LCH, to uniform the treatment of adult patients with LCH and to improve treatment results with respect to survival, therapy response, prevention of…
ID
Source
Brief title
Condition
- White blood cell disorders
- Haematopoietic neoplasms (excl leukaemias and lymphomas)
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
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 outcome
Not applicable.
Background summary
Langerhans Cell Histiocytosis is a rare, tumor-like disease characterized by
the dysregulated growth, activity and trafficking of Langerhans cells. It has
an unpredictable course and can be fatal. The cause of the disease is unknown.
The disease has been better recognized in children and thus most of the
available information concerning clinical features, pathogenesis and treatment
derives from the pediatric experience.
Limited experience is avalaible so far on LCH in adult patients.
Study objective
To define and implement an uniform initial evaluation and stratification on
adult patients with LCH, to uniform the treatment of adult patients with LCH
and to improve treatment results with respect to survival, therapy response,
prevention of disease recurrence and late effects.
Study design
The study is an intervention study and for patients in group 2, the duration of
treatment will be the object of a randomized study, 6 months versus 12 months.
Intervention
Group 1: treatment with Prednisone, Vinblastine and Mercaptopurine.
Prednisone is given orally as tablets, daily during the initial 6 weeks, then
as 5 daily pulses every 3 weeks during the continuation treatment.
Mecaptopurine is given as daily oral tablets during the continuation therapy.
Vinblastine is given intravenously as an injection weekly the initial 6 weeks,
and every 3 weeks during the continuation treatment.
Total duration of treatment is 6 months.
Group 2: same as group 1.
Total of duration of treatment will be the object of a randomized study: 6
months versus 12 months
Group 3: an observational phase of 6 months after cigarette smoke withdrawal.
In case of progression of the symptoms or pulmonary dysfunction, treatment
phase starts: steroid monotherapy with Prednisone for 6 months.
Study burden and risks
The treatment requires frequent visits to the hospital for administration of
the chemotherapy as well as monitoring for potential complications as is
normally required for all other regular/standard systemic treatment.
Side effects of the treatment with Prednisone, and/or Vinblastine and
Mercaptopurine are already known, but will appear.
Benefit to be gained from participation in this research study is control of
the disease. Information will be gained that will be useful to researchers
studying the disease.
Postbus 7057
1007 MB Amsterdam
NL
Postbus 7057
1007 MB Amsterdam
NL
Listed location countries
Age
Inclusion criteria
- definitive diagnosis of LCH
- no prior cytoreductive treatment for LCH
Exclusion criteria
- patients with severe impairment of clinical condition including severely impaired pulmonary function, long term oxygen therapy or cor pulmonale.
- treatment with immune suppressive agents and/or biphosphonates within 4 weeks from baseline evaluation
- pregnancy
Design
Recruitment
Medical products/devices used
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| EudraCT | EUCTR2006-002392-40-NL |
| CCMO | NL13165.029.07 |