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Busulfan, Cyclophosphamide, and Melphalan or Busulfan and Fludarabine Phosphate Before Donor Hematopoietic Cell Transplant in Treating Younger Patients With Juvenile Myelomonocytic Leukemia

Phase 2
Completed
Conditions
Juvenile Myelomonocytic Leukemia
Interventions
Procedure: Allogeneic Hematopoietic Stem Cell Transplantation
Other: Laboratory Biomarker Analysis
Other: Pharmacological Study
Registration Number
NCT01824693
Lead Sponsor
Children's Oncology Group
Brief Summary

This randomized phase II trial studies how well giving busulfan, cyclophosphamide, and melphalan or busulfan and fludarabine phosphate before donor hematopoietic cell transplant works in treating younger patients with juvenile myelomonocytic leukemia. Giving chemotherapy before a donor hematopoietic transplant helps stop the growth of cancer cells. It may also stop the patient's immune system from rejecting the donor's stem cells. When the healthy stem cells from a donor are infused into the patient, they may help the patient's bone marrow make stem cells, red blood cells, white blood cells, and platelets. It is not yet known whether giving busulfan, cyclophosphamide, and melphalan or busulfan and fludarabine phosphate before a donor stem cell transplant is more effective in treating juvenile myelomonocytic leukemia.

Detailed Description

PRIMARY OBJECTIVES:

I. To compare ? in a randomized fashion ? the day 100 treatment related mortality (TRM) incidence for two myeloablative conditioning regimens, busulfan-fludarabine (fludarabine phosphate) (BU-FLU) and busulfan-cyclophosphamide-melphalan (BU-CY-MEL), prior to hematopoietic cell transplant (HCT) for children with juvenile myelomonocytic leukemia (JMML), in order to determine the preferred regimen for future trials.

II. To compare ? in a randomized fashion ? the 18-month event-free survival (EFS) following two different myeloablative conditioning regimens (BU-FLU vs. BU-CY-MEL) prior to HCT for children with JMML, in order to determine the preferred regimen for future trials.

SECONDARY OBJECTIVES:

I. To determine the 18-month relapse incidence (RI) following two different myeloablative conditioning regimens (BU-FLU vs. BU-CY-MEL) prior to HCT for children with JMML.

II. To determine the graft failure rates following two different myeloablative conditioning regimens (BU-FLU vs. BU-CY-MEL) prior to HCT for children with JMML.

TERTIARY OBJECTIVES:

I. To determine the rates of severe toxicities (grade 3/4) at day 100 post-HCT between the two myeloablative conditioning regimens (BU-FLU vs. BU-CY-MEL).

II. To determine the rates of acute and chronic (at 18 months post-HCT) graft-versus-host disease (GVHD) following HCT using two different conditioning regimens (BU-FLU vs. BU-CY-MEL) in children with JMML.

III. To create a JMML-specific pre-HCT index to allow better risk-stratification of future patients.

IV. To determine the feasibility of assessing post-transplant disease burden by donor chimerism measurements and allele-specific polymerase chain reaction (PCR) in mononuclear and sorted cell subsets.

V. To validate gene expression and methylation classifiers predictive of relapse in patients with JMML.

VI. To comprehensively assess genetic and biochemical alterations amongst patients with JMML who are treated on this transplant protocol.

OUTLINE: Patients are randomized to 1 of 2 treatment arms.

ARM I:

CONDITIONING REGIMEN: Patients receive busulfan intravenously (IV) over 2-3 hours once daily (QD), every 12 hours, or every 6 hours on days -8 to -5, cyclophosphamide IV over 60 minutes QD on days -4 and -3, and melphalan IV over 15-30 minutes on day -1.

TRANSPLANT: Patients undergo allogeneic HCT on day 0.

Patients receive tacrolimus IV or orally (PO) on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).

ARM II:

CONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 30-60 minutes on days -5 to -2.

TRANSPLANT: Patients undergo allogeneic HCT as in Arm I.

Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).

After completion of study treatment, patients are followed up for 5 years.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
30
Inclusion Criteria
  • Patients must have a strong clinical suspicion of JMML, based on a modified category 1 of the revised diagnostic criteria; specifically, eligible patients must have all of the following:

    • Splenomegaly
    • Absolute monocyte count (AMC) > 1000/uL
    • Blasts in peripheral blood (PB)/bone marrow (BM) < 20%
  • For the 7-10% of patients without splenomegaly, the diagnostic entry criteria must include all other features described above and at least 2 of the following criteria:

    • Circulating myeloid precursors
    • White blood cell (WBC) > 10,000/uL
    • Increased fetal hemoglobin (HgbF) for age
    • Sargramostim (GM-CSF) hypersensitivity OR, patients must have been previously diagnosed with JMML
  • Patients must be previously untreated with HCT

  • All patients and/or their parents or legal guardians must sign a written informed consent

  • All institutional, Food and Drug Administration (FDA), and National Cancer Institute (NCI) requirements for human studies must be met

Exclusion Criteria
  • Patients with a known germline mutation of PTPN11 (Noonan?s Syndrome) are not eligible

  • Patients with a known history of NF1 (Neurofibromatosis Type 1) and either

    • A history of a tumor of the central nervous system (astrocytoma or optic glioma), or
    • A malignant peripheral nerve sheath tumor with a complete remission of < 1 year are not eligible
  • Human immunodeficiency virus (HIV) positive patients are not eligible

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Arm I (busulfan, cyclophosphamide, melphalan)Laboratory Biomarker AnalysisCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)Allogeneic Hematopoietic Stem Cell TransplantationCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)Pharmacological StudyCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)Pharmacological StudyCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)BusulfanCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)Laboratory Biomarker AnalysisCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)MelphalanCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)Allogeneic Hematopoietic Stem Cell TransplantationCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)BusulfanCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)CyclophosphamideCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)Mycophenolate MofetilCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm I (busulfan, cyclophosphamide, melphalan)TacrolimusCONDITIONING REGIMEN: Patients receive busulfan IV QD, every 12 hours, or every 6 hours over 2-3 hours on days -8 to -5, cyclophosphamide IV QD over 60 minutes on days -4 and -3, and melphalan IV over 15-30 minutes on day -1. TRANSPLANT: Patients undergo allogeneic HCT no sooner than 24 hours after the last dose of chemotherapy. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)Fludarabine PhosphateCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)Mycophenolate MofetilCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Arm II (busulfan, fludarabine phosphate)TacrolimusCONDITIONING REGIMEN: Patients receive busulfan as in Arm I and fludarabine phosphate IV over 1 hour on days -5 to -2. TRANSPLANT: Patients undergo allogeneic HCT as in Arm I. Patients receive tacrolimus IV or PO on days -1 to 98 (related donor) or 180 (unrelated donor) and mycophenolate mofetil IV over 2 hours or PO every 8 hours on days 1-30 (related donor) or 45 (unrelated donor).
Primary Outcome Measures
NameTimeMethod
Number of Participants Who Experience Treatment-Related Mortality (TRM) by Day 100From transplant up to 100 days

The number of patients who experience TRM on day 100. Treatment-Related Mortality (TRM) an event defined as a death prior to relapse or non-response. Time to TRM is defined as time from transplants to TRM. Patients who die between the start of the conditioning regimen and transplant will be considered a TRM with time to TRM of zero.

Percent Probability of Event-free Survival (EFS)From transplant up to 18 months

Probability of Event-free Survival (EFS) for Patients after 18 months. An event is either treatment related mortality (TRM), primary or secondary graft failure, or relapse/non-response (as defined in protocol section 10). Time to event is time from transplant with patients who die between the start of the conditioning regimen and transplant given a time to event of zero.

Secondary Outcome Measures
NameTimeMethod
Percentage of Participants Who Experience Primary Graft Failure Event Between ArmsDay 0 - day 540 (18 months) following completion of stem cell transplant

Primary Graft failure is defined as the failure to achieve an ANC \>= 500/uL after 42 days, determined by 3 consecutive measurements on different days; OR \< 5% donor cells in blood or bone marrow by day +42 (as demonstrated by a chimerism assay), without evidence of Juvenile Myelomonocytic Leukemia (JMML).

Percent Probability of 18 Months-relapse Event Between ArmsFrom transplant up to 18 months

Probability of patients relapsing at 18 months. A relapse event is defined in protocol section 10.2.3. Time to relapse/non-response is defined as time from transplant to when all criteria of section 10.2.3 are met.

Trial Locations

Locations (50)

Rainbow Babies and Childrens Hospital

🇺🇸

Cleveland, Ohio, United States

Mattel Children's Hospital UCLA

🇺🇸

Los Angeles, California, United States

City of Hope Comprehensive Cancer Center

🇺🇸

Duarte, California, United States

Children's Hospital of Alabama

🇺🇸

Birmingham, Alabama, United States

Rady Children's Hospital - San Diego

🇺🇸

San Diego, California, United States

UCSF Medical Center-Parnassus

🇺🇸

San Francisco, California, United States

UCSF Medical Center-Mission Bay

🇺🇸

San Francisco, California, United States

Lurie Children's Hospital-Chicago

🇺🇸

Chicago, Illinois, United States

Children's Healthcare of Atlanta - Egleston

🇺🇸

Atlanta, Georgia, United States

Riley Hospital for Children

🇺🇸

Indianapolis, Indiana, United States

University of Iowa/Holden Comprehensive Cancer Center

🇺🇸

Iowa City, Iowa, United States

Norton Children's Hospital

🇺🇸

Louisville, Kentucky, United States

Dana-Farber Cancer Institute

🇺🇸

Boston, Massachusetts, United States

University of Minnesota/Masonic Cancer Center

🇺🇸

Minneapolis, Minnesota, United States

The Childrens Mercy Hospital

🇺🇸

Kansas City, Missouri, United States

Cardinal Glennon Children's Medical Center

🇺🇸

Saint Louis, Missouri, United States

Montefiore Medical Center - Moses Campus

🇺🇸

Bronx, New York, United States

Columbia University/Herbert Irving Cancer Center

🇺🇸

New York, New York, United States

New York Medical College

🇺🇸

Valhalla, New York, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

University of Oklahoma Health Sciences Center

🇺🇸

Oklahoma City, Oklahoma, United States

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

Children's Hospital of Pittsburgh of UPMC

🇺🇸

Pittsburgh, Pennsylvania, United States

Medical City Dallas Hospital

🇺🇸

Dallas, Texas, United States

UT Southwestern/Simmons Cancer Center-Dallas

🇺🇸

Dallas, Texas, United States

Cook Children's Medical Center

🇺🇸

Fort Worth, Texas, United States

Methodist Children's Hospital of South Texas

🇺🇸

San Antonio, Texas, United States

University of Wisconsin Hospital and Clinics

🇺🇸

Madison, Wisconsin, United States

Seattle Children's Hospital

🇺🇸

Seattle, Washington, United States

Phoenix Childrens Hospital

🇺🇸

Phoenix, Arizona, United States

Alfred I duPont Hospital for Children

🇺🇸

Wilmington, Delaware, United States

Nemours Children's Clinic-Jacksonville

🇺🇸

Jacksonville, Florida, United States

Johns Hopkins All Children's Hospital

🇺🇸

Saint Petersburg, Florida, United States

University of Nebraska Medical Center

🇺🇸

Omaha, Nebraska, United States

Johns Hopkins University/Sidney Kimmel Cancer Center

🇺🇸

Baltimore, Maryland, United States

Wayne State University/Karmanos Cancer Institute

🇺🇸

Detroit, Michigan, United States

Hackensack University Medical Center

🇺🇸

Hackensack, New Jersey, United States

Medical University of South Carolina

🇺🇸

Charleston, South Carolina, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Oregon Health and Science University

🇺🇸

Portland, Oregon, United States

British Columbia Children's Hospital

🇨🇦

Vancouver, British Columbia, Canada

Primary Children's Hospital

🇺🇸

Salt Lake City, Utah, United States

The Montreal Children's Hospital of the MUHC

🇨🇦

Montreal, Quebec, Canada

Centre Hospitalier Universitaire Sainte-Justine

🇨🇦

Montreal, Quebec, Canada

Starship Children's Hospital

🇳🇿

Grafton, Auckland, New Zealand

CancerCare Manitoba

🇨🇦

Winnipeg, Manitoba, Canada

Princess Margaret Hospital for Children

🇦🇺

Perth, Western Australia, Australia

University of Rochester

🇺🇸

Rochester, New York, United States

University of Mississippi Medical Center

🇺🇸

Jackson, Mississippi, United States

Children's Hospital of Orange County

🇺🇸

Orange, California, United States

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