Hemophagocytic Lymphohistiocytosis (HLH), a life-threatening disease usually occurring in early childhood. HLH-2004 is an international treatment protocol which will be compared with the previous HLH-94 protocol. In HLH-2004 the cyclosporine treatment will be introduced at the start of therapy instead of after 8 weeks. Intrathecal therapy will consist of methotrexate in combination with prednisolone instead of methotrexate alone. Only a minority of the patients will receive intrathecal therapy.
Conditions
Interventions
Trade Name: Several
Product Name: etoposide
Pharmaceutical Form: Injection*
INN or Proposed INN: etoposide
Concentration unit: mg milligram(s)
Concentration type: equal
Product Name: dexamethasone
Ph
Sponsors
Barncancerforskningsenheten, Astrid Lindgrens Barnsjukhus
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: The diagnosis of HLH. All patients with this diagnosis will be offered the treatment protocol. All patients diagnosed with the disease for the first time are eligible for inclusion. Are the trial subjects under 18? yes Number of subjects for this age range: F.1.2 Adults (18-64 years) no F.1.2.1 Number of subjects for this age range F.1.3 Elderly (>=65 years) no F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria:
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: To improve survival and reduce the prevalence of neurological sequelae. HLH-94 achieved a survival before stem cell transplantation (SCT) of 75 %, and in the present protocol we aim at improving the survival before SCT to 85 %.;Secondary Objective: Survival and long-term outcome after stem cell transplantation. Genetic characterization of familial cases.;Primary end point(s): Survival before stem cell transplantation, total survival, and the prevalence of neurological sequelae | — |
Countries
Norway
Outcome results
None listed