Treatment Protocol for Hemophagocytic Lymphohistiocytosis 2004
NCT ID: NCT00426101
Last Updated: 2018-07-05
Study Results
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Basic Information
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COMPLETED
PHASE3
368 participants
INTERVENTIONAL
2004-01-31
2017-12-31
Brief Summary
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Detailed Description
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Conditions
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Study Design
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NA
SINGLE_GROUP
TREATMENT
NONE
Study Groups
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Etoposide, Dexamethasone, Cyclosporin A plus IT MTX & Steroids
As compared to the HLH-94 treatment, the main changes are that
1. Cyclosporin A is administered from day 1 and
2. Intrathecal steroids are added to the intrathecal methotrexate.
Drugs, dosage, frequency and duration are described in the paragraph "Interventions" below.
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
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
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.
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.
Stem cell transplant
The SCT procedure is up to the treating physician. However, a suggested regimen is provided.
Interventions
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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
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
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.
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.
Stem cell transplant
The SCT procedure is up to the treating physician. However, a suggested regimen is provided.
Eligibility Criteria
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Inclusion Criteria
Exclusion Criteria
18 Years
ALL
No
Sponsors
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Meyer Children's Hospital IRCCS
OTHER
Leiden University Medical Center
OTHER
Children's Hospital Medical Center, Cincinnati
OTHER
Ehime University Graduate School of Medicine
OTHER
Universitätsklinikum Hamburg-Eppendorf
OTHER
Baylor College of Medicine
OTHER
Great Ormond Street Hospital for Children NHS Foundation Trust
OTHER
St. Anna Kinderkrebsforschung
OTHER
Hospital de Cruces
OTHER
Hospital JP Garrahan
OTHER_GOV
Karolinska University Hospital
OTHER
Responsible Party
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Jan-Inge Henter
Professor
Principal Investigators
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Jan-Inge Henter, MD, PhD
Role: PRINCIPAL_INVESTIGATOR
Karolinska Institutet
Locations
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Childhood Cancer Research Unit, Karolinska Hospital
Stockholm, , Sweden
Countries
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References
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Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124-31. doi: 10.1002/pbc.21039.
Bergsten E, Horne A, Arico M, Astigarraga I, Egeler RM, Filipovich AH, Ishii E, Janka G, Ladisch S, Lehmberg K, McClain KL, Minkov M, Montgomery S, Nanduri V, Rosso D, Henter JI. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood. 2017 Dec 21;130(25):2728-2738. doi: 10.1182/blood-2017-06-788349. Epub 2017 Sep 21.
Bergsten E, Horne A, Hed Myrberg I, Arico M, Astigarraga I, Ishii E, Janka G, Ladisch S, Lehmberg K, McClain KL, Minkov M, Nanduri V, Rosso DA, Sieni E, Winiarski J, Henter JI. Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: results from the HLH-2004 study. Blood Adv. 2020 Aug 11;4(15):3754-3766. doi: 10.1182/bloodadvances.2020002101.
Other Identifiers
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HLH-2004
Identifier Type: -
Identifier Source: org_study_id
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