NCT00426101

Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004

HLH-2004 Treatment Protocol

• Status: Completed
• Phase: Phase 3
• Study type: Interventional
• Enrollment: 368 (actual)
• Sponsor: Karolinska University Hospital · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Not accepted

Summary

Without therapy HLH is often fatal, and often rapidly fatal. The treatment protocol HLH-94 has improved survival markedly as compared to the survival earlier. We now aim to improve survival further.

Detailed description

The most dangerous period after HLH diagnosis is the first 2 months. In HLH-2004 we provide additional therapy during this period as compared to in HLH-94.

Conditions

• Hemophagocytic Lymphohistiocytosis

Interventions

Type	Name	Description
DRUG	Dexamethasone	10 mg/m2 daily wk 1-2 5 mg/m2 daily wk 3-4 2.5 mg/m2 daily wk 5-6 1.25 mg/m2 daily wk 7 Steroids tapered wk 8 If continuation: Pulses every 2nd wk, 10 mg/m2 for 3 days
DRUG	Etoposide	150 mg/m2 iv twice/wk (wk 1-2) 150 mg/m2 iv once/wk (wk 3-8) If continuation: 150 mg/m2 iv, every 2nd wk
DRUG	Cyclosporin	WK 1-8: \- Aim at around 200 microgram/L (trough value). Start: 6 mg/kg daily (divided in 2 daily doses) wk 1, if kidney function is normal. If continuation: \- Aim for around 200 microgram/L. Monitor GFR.
PROCEDURE	Intrathecal therapy	If at 2 wks there are progressive neurological symptoms or if an abnormal CSF (cell count and protein) has not improved, then give 4 wkly intrathecal inj. Be aware that some pat may have increased intracranial pressure. Methotrexate: \<1 yr 6 mg, 1-2 yrs 8 mg, 2-3 yrs 10 mg, \>3 yrs 12 mg. Prednisolone: \<1 yr 4 mg, 1-2 yrs 6 mg, 2-3 yrs 8 mg, \>3 yrs 10 mg.
PROCEDURE	Stem cell transplant	The SCT procedure is up to the treating physician. However, a suggested regimen is provided.

Timeline

• Start date: 2004-01-01
• Primary completion: 2011-12-01
• Completion: 2017-12-01
• First posted: 2007-01-24
• Last updated: 2018-07-05

Locations

1 site across 1 country: Sweden