Status:

COMPLETED

Bone Marrow Cell Gene Transfer in Individuals With Fanconi Anemia

Lead Sponsor:

Boston Children's Hospital

Collaborating Sponsors:

National Heart, Lung, and Blood Institute (NHLBI)

Children's Hospital Medical Center, Cincinnati

Conditions:

Fanconi Anemia

Eligibility:

All Genders

1-35 years

Phase:

PHASE1

Brief Summary

Fanconi anemia (FA) is a disease that affects an individual's bone marrow. It is caused by a defective gene in the bone marrow cells that produce various types of blood cells. Individuals with FA may ...

Detailed Description

FA is a rare, inherited disease that is caused by a gene defect and that primarily affects an individual's bone marrow, resulting in decreased production of blood cells. The lack of white blood cells ...

Eligibility Criteria

Inclusion

  • FA, as determined by a positive test for increased sensitivity to chromosomal breakage with mitomycin C or diepoxybutane
  • FA complementation group A, as determined by somatic cell hybrids or molecular characterization; transduction of peripheral blood or bone marrow cells with the complementation group of specific retrovirus used in this study must demonstrate correction of mitomycin C sensitivity or cell cycle arrest
  • Weighs at least 7.5 kg
  • Normal cytogenetics on bone marrow within 3 months of study entry
  • A minimum of 2 x 10(6) CD34+ cells/kg after CD34+ selection of the harvested bone marrow or mobilized peripheral blood product must be available to proceed with thaw (if cryopreserved) and transduction
  • Human leukocyte antigen (HLA) typing with initial donor limited search results that indicate a potentially acceptable matched unrelated donor in the National Marrow Donor Program database

Exclusion

  • Cancer
  • Clonal cytogenetic abnormality on bone marrow or peripheral blood karyotype within 3 months of study entry
  • Myelodysplastic syndrome based on the FAB classification including:
  • Refractory anemia with ringed sideroblasts (RARS)
  • Refractory anemia with excess blasts (RAEB)
  • RAEB in transformation (RAEB-T)
  • Chronic myelomonocytic leukemia (CMML) (myelodysplastic changes in greater than two cell lines, refractory anemia alone, or aplastic anemia with dysplastic changes are permitted)
  • Positive baseline screening result for both of the following:
  • Detection of Fanconi A proviral sequences by polymerase chain reaction (PCR) analysis
  • Detection of replication competent retrovirus by repeat testing by PCR of gibbon ape leukemia virus (GALV) envelope sequence or a positive S+L- assay
  • Pregnant or breastfeeding; women of childbearing potential who are enrolled will be advised that the drug may cause birth defects and will be required to use an acceptable form of contraception
  • Concurrent enrollment in any other study using an investigational agent, excluding androgens and thyroxine
  • Physical or emotional status that would prevent informed consent, protocol compliance, or adequate follow-up with participant or legal guardian
  • Participants for whom an acceptable HLA identical matched sibling donor (HLA A, B, DRB1; 6/6 match) has been identified (HLA typing of normal siblings must be documented)

Key Trial Info

Start Date :

August 1 2004

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

October 1 2007

Estimated Enrollment :

3 Patients enrolled

Trial Details

Trial ID

NCT00272857

Start Date

August 1 2004

End Date

October 1 2007

Last Update

June 23 2017

Active Locations (1)

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1

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States, 45229