Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004

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.

Conditions

• Hemophagocytic Lymphohistiocytosis

Interventions

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.

Sponsors & Collaborators

• Meyer Children's Hospital IRCCS

  collaborator OTHER

• Leiden University Medical Center

  collaborator OTHER

• Children's Hospital Medical Center, Cincinnati

  collaborator OTHER

• Ehime University Graduate School of Medicine

  collaborator OTHER

• Universitätsklinikum Hamburg-Eppendorf

  collaborator OTHER

• Baylor College of Medicine

  collaborator OTHER

• Great Ormond Street Hospital for Children NHS Foundation Trust

  collaborator OTHER

• St. Anna Kinderkrebsforschung

  collaborator OTHER

• Hospital de Cruces

  collaborator OTHER

• Hospital JP Garrahan

  collaborator OTHER_GOV

• Karolinska University Hospital

  lead OTHER

Principal Investigators

• Jan-Inge Henter, MD, PhD · Karolinska Institutet

Study Design

Allocation

NA

Purpose

TREATMENT

Masking

NONE

Model

SINGLE_GROUP

Eligibility

Max Age

18 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2004-01-31

Primary Completion

2011-12-31

Completion

2017-12-31

Countries

• Sweden

Study Locations