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NCT Number: NCT00426101

Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004

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.

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Key information

Conditions

Hemophagocytic Lymphohistiocytosis Hemic and Lymphatic Diseases Histiocytosis Histiocytosis, Non-Langerhans-Cell Lymphatic Diseases Lymphohistiocytosis, Hemophagocytic

Age range

Up to 18 year

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 3

Primary location

Childhood Cancer Research Unit, Karolinska Hospital

Stockholm, S-171 76, Sweden

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Patients who fulfil the diagnostic criteria of HLH.

Exclusion criteria

  • Prior cytotoxic or cyclosporin treatment for HLH.

Treatment and study plan

Dexamethasone

Drug

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

etoposide

Drug

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

Cyclosporin

Drug

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.

Intrathecal therapy

Procedure

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.

Stem Cell Transplant

Procedure

The SCT procedure is up to the treating physician. However, a suggested regimen is provided.

Primary outcomes

  1. Survival

    Time frame: 1-year after diagnosis

Secondary outcomes

  1. Late effects

    Time frame: 5-years after diagnosis

Sponsors and collaborators

Lead sponsor

Karolinska University Hospital

Other

Collaborators

  • Baylor College of Medicine
  • Children's Hospital Medical Center, Cincinnati
  • Ehime University Graduate School of Medicine
  • Great Ormond Street Hospital for Children NHS Foundation Trust
  • Hospital JP Garrahan
  • Hospital de Cruces
  • Leiden University Medical Center
  • Meyer Children's Hospital IRCCS
  • St. Anna Kinderkrebsforschung
  • Universitätsklinikum Hamburg-Eppendorf

Registry information

Official study title

HLH-2004 Treatment Protocol

Important dates

Study start
2004
Primary completion
2011
Study completion
2017
First posted
Jan 24, 2007
Registry last updated
Jul 5, 2018

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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