NCT01104025

Brief Summary

Despite good progress during the last decade, hemophagocytic lymphohistiocytosis (HLH) remains difficult to treat. Two different treatment regimens have been used successfully. The first one, a treatment regimen based on two drugs called etoposide and dexamethasone, has been used worldwide. The second regimen, based on two drugs called Anti-thymocyte globulin (ATG) and prednisone, has been used mostly at one hospital in Paris, for over 15 years. With either regimen, about three quarters of treated children survive the most difficult time, the first two months after diagnosis. These two different regimens appear to work somewhat differently, and we suspect that combining them may give better results than either regimen alone. We are conducting this clinical trial to test the combination of ATG, dexamethasone, and etoposide for the treatment of HLH. The purpose of this research study is to find out what effects (good and bad) this drug combination has on you and your HLH.

Trial Health

90
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
31

participants targeted

Target at P25-P50 for phase_2

Timeline
Completed

Started Apr 2010

Longer than P75 for phase_2

Geographic Reach
2 countries

15 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

April 1, 2010

Completed
12 days until next milestone

First Submitted

Initial submission to the registry

April 13, 2010

Completed
2 days until next milestone

First Posted

Study publicly available on registry

April 15, 2010

Completed
5.6 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

November 1, 2015

Completed
5 months until next milestone

Study Completion

Last participant's last visit for all outcomes

April 1, 2016

Completed
4.6 years until next milestone

Results Posted

Study results publicly available

October 19, 2020

Completed
Last Updated

October 19, 2020

Status Verified

September 1, 2020

Enrollment Period

5.6 years

First QC Date

April 13, 2010

Results QC Date

August 28, 2020

Last Update Submit

September 21, 2020

Conditions

Keywords

hemophagocytic lymphohistiocytosishybrid immunotherapydexamethasoneEtoposideATG,rabbit

Outcome Measures

Primary Outcomes (1)

  • Overall Survival

    To determine the overall survival of patients with hemophagocytic lymphohistiocytosis at 8 weeks after an ATG/etoposide-based induction regimen and to determine the feasibility of this approach in the context of a multicenter clinical trial.

    8 Weeks

Secondary Outcomes (5)

  • Time to Response

    8 Weeks

  • Overall Survival

    up to day 180

  • Number of Participants Who Experienced Reactivation

    up to 180 days

  • Overall Survival to Day +100

    up to day 280

  • Disease Status at BMT

    up to day 180

Study Arms (1)

Induction Therapy

EXPERIMENTAL

ATG, rabbit: intravenous, 5 mg/kg/dose, 5 consecutive days Dexamethasone: intravenous, 20mg/m2/day x7days, 10mg/m2/day x7days, 5mg/m2/day x14days, 2.5mg/m2/day x14days, 1.25mg/m2/day x14days Etoposide: intravenous, 150 mg/m2 weekly, starting 7 days after first dose of Thymoglobulin Methotrexate and hydrocortisone: intrathecal to patients with central nervous system involvement, age\< 1 yr: 6/8mg (MTX/HC), 1-2 yrs: 8/10mg, 2-3 yrs: 10/12mg, \>3 yrs: 12/15 mg, on day 7, 14, 21 and 42

Drug: ATG, rabbitDrug: EtoposideDrug: MethotrexateDrug: hydrocortisoneDrug: Dexamethasone

Interventions

ATG, rabbit (Thymoglobulin, Genzyme) will be dosed at 5 mg/kg/dose, given IV on 5 consecutive days (titrated over 4 to 8 hours).

Also known as: Thymoglobulin
Induction Therapy

Etoposide will be dosed at 150mg/m2, given IV. The first dose will be given 7 days (+/- 2 days) after the first dose of ATG, and be given weekly for a total of 7 doses.

Also known as: Etopophos, Toposar, VePesid
Induction Therapy

Intrathecal Methotrexate and hydrocortisone will be administered to CNS+ patients (CNS+ patients are those patients which have any of the following: elevated CSF (cerebral spinal fluid) protein or white count, seizures, focal or global neurologic deficit, MRI abnormalities consistent with CNS involvement by HLH.) in the following doses: age\< 1 yr: 6/8mg (MTX/HC), 1-2 yrs: 8/10mg, 2-3 yrs: 10/12mg, \>3 yrs: 12/15 mg. It will be administered (+/- 3 days) on day 7, 14, 21 and 42.

Induction Therapy

Intrathecal Methotrexate and hydrocortisone will be administered to CNS+ patients (CNS+ patients are those patients which have any of the following: elevated CSF (cerebral spinal fluid) protein or white count, seizures, focal or global neurologic deficit, MRI abnormalities consistent with CNS involvement by HLH.) in the following doses: age\< 1 yr: 6/8mg (MTX/HC), 1-2 yrs: 8/10mg, 2-3 yrs: 10/12mg, \>3 yrs: 12/15 mg. It will be administered (+/- 3 days) on day 7, 14, 21 and 42.

Induction Therapy

will be started with the ATG. It will be divided BID, given IV for at least 1 week before switching to PO. Dosing: 20mg/m2/day x7days, 10mg/m2/day x7days, 5mg/m2/day x14days, 2.5mg/m2/day x14days, 1.25mg/m2/day x14days.

Induction Therapy

Eligibility Criteria

AgeUp to 18 Years
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64)

You may qualify if:

  • diagnosis of hemophagocytic lymphohistiocytosis
  • Patients \<18 years of age
  • The patient must have active disease at the time of enrollment
  • Patient's legal guardians must sign an Institutional Review Board approved consent form indicating their awareness of the investigational nature and the risks of this study.
  • Eligible subjects must be enrolled with the protocol coordinating center

You may not qualify if:

  • Recent treatment, within 3 months, with another therapeutic regimen for HLH
  • Known active malignancy
  • Known rheumatologic diagnosis which may be the underlying cause of HLH
  • Pregnancy (as determined by serum or urine test) or active breast feeding
  • Failure to provide signed informed consent

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (15)

Phoenix Children's Hospital

Phoenix, Arizona, 85254, United States

Location

University of California, San Francisco Department of Pediatrics

San Francisco, California, 94143, United States

Location

Stanford University

Stanford, California, 94305, United States

Location

University of Colorado

Aurora, Colorado, 80045, United States

Location

Nemours

Wilmington, Delaware, 19803, United States

Location

Children's National Medical Center

Washington D.C., District of Columbia, 20010, United States

Location

Nemours

Jacksonville, Florida, 32827, United States

Location

Florida All Children's Hospital

St. Petersburg, Florida, 33701, United States

Location

Tulane University Medical Center

New Orleans, Louisiana, 70118, United States

Location

Children's Hospital Boston

Boston, Massachusetts, 02115, United States

Location

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, 45229, United States

Location

Oregon Health and Science University

Portland, Oregon, 21703, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19145, United States

Location

Texas Children's Cancer Center/Baylor College of Medicine

Houston, Texas, 77030, United States

Location

The Hospital for Sick Children

Toronto, Ontario, Canada

Location

MeSH Terms

Conditions

Lymphohistiocytosis, Hemophagocytic

Interventions

thymoglobulinEtoposideetoposide phosphateMethotrexateHydrocortisoneDexamethasone

Condition Hierarchy (Ancestors)

Histiocytosis, Non-Langerhans-CellHistiocytosisLymphatic DiseasesHemic and Lymphatic Diseases

Intervention Hierarchy (Ancestors)

PodophyllotoxinTetrahydronaphthalenesNaphthalenesPolycyclic Aromatic HydrocarbonsHydrocarbons, AromaticHydrocarbons, CyclicHydrocarbonsOrganic ChemicalsPolycyclic CompoundsGlucosidesGlycosidesCarbohydratesAminopterinPterinsPteridinesHeterocyclic Compounds, 2-RingHeterocyclic Compounds, Fused-RingHeterocyclic CompoundsPregnenedionesPregnenesPregnanesSteroidsFused-Ring Compounds11-HydroxycorticosteroidsHydroxycorticosteroidsAdrenal Cortex HormonesHormonesHormones, Hormone Substitutes, and Hormone Antagonists17-HydroxycorticosteroidsPregnadienetriolsPregnadienesSteroids, Fluorinated

Limitations and Caveats

This is a single arm study.

Results Point of Contact

Title
Michael Jordan, MD
Organization
Cincinnati Children's Hospital Medical Center

Study Officials

  • Michael Jordan, MD

    Children's Hospital Medical Center, Cincinnati

    PRINCIPAL INVESTIGATOR

Publication Agreements

PI is Sponsor Employee
Yes

Study Design

Study Type
interventional
Phase
phase 2
Allocation
NA
Masking
NONE
Purpose
TREATMENT
Intervention Model
SINGLE GROUP
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

April 13, 2010

First Posted

April 15, 2010

Study Start

April 1, 2010

Primary Completion

November 1, 2015

Study Completion

April 1, 2016

Last Updated

October 19, 2020

Results First Posted

October 19, 2020

Record last verified: 2020-09

Locations