Status:

COMPLETED

TBI Dose De-escalation for Fanconi Anemia

Lead Sponsor:

Masonic Cancer Center, University of Minnesota

Conditions:

Fanconi Anemia

Eligibility:

All Genders

Phase:

PHASE2

PHASE3

Brief Summary

This is a single arm, total body irradiation (TBI) trial. All patients will be prescribed TBI 300 cGy with the goal of evaluating secondary endpoints.

Detailed Description

Study Treatment: Patients will receive voriconazole (antifungal therapy) by mouth beginning 1 month prior to conditioning therapy, if possible. 1) The subject is to receive total body irradiation (300...

Eligibility Criteria

Inclusion

  • Meeting the definition of standard risk or high risk Fanconi anemia as defined in the next two sections:
  • Standard risk patients must be \<18 years of age with a diagnosis of Fanconi anemia with aplastic anemia (AA), myelodysplastic syndrome without excess blasts, or high risk genotype as defined below:
  • Aplastic anemia is defined as having at least one of the following when not receiving growth factors or transfusions:
  • platelet count \<20 \* 10\^9/L
  • ANC \<5 \* 10\^8/L
  • Hemoglobin \<8 g/dL
  • Myelodysplastic syndrome (MDS) with multilineage dysplasia with or without chromosomal anomalies
  • High risk genotype (e.g. IVS-4 or exon 14 FANCC mutations, or BRCA1 or 2 mutations)
  • High risk patients must have one or more of the following high risk features:
  • Advanced MDS (≥ 5% blast) or acute leukemia
  • Require additional HSCT for graft failure
  • History at any time of systemic fungal or gram negative infection
  • Severe renal disease with a creatinine clearance \<40 mL/min
  • Age \> 18 years
  • Very high risk patients must have Advanced MDS (≥ 5% blast) or acute leukemia after initial hematopoietic stem cell transplant (HSCT)
  • Patients must have an appropriate source of stem cells. Patients and donors will be typed for HLA-A, B, C and DRB1 using high resolution molecular typing.
  • Adequate major organ function including:
  • Cardiac: ejection fraction \>45%
  • Hepatic: bilirubin, AST or ALT, ALP \<5 x normal
  • Karnofsky performance status \>70% or Lansky \>50 (if \< 16 years of age)
  • Women of child-bearing age must be using adequate birth control and have a negative pregnancy test.
  • Written consent.

Exclusion

  • Available HLA-genotypically identical related donor in standard risk patients.
  • Active central nervous system (CNS) leukemia at time of study enrollment.
  • History of squamous cell carcinoma of the head/neck/cervix within previous 2 years.
  • Prior radiation therapy that prevents further total body irradiation (TBI).

Key Trial Info

Start Date :

May 18 2006

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

October 9 2020

Estimated Enrollment :

83 Patients enrolled

Trial Details

Trial ID

NCT00352976

Start Date

May 18 2006

End Date

October 9 2020

Last Update

November 24 2021

Active Locations (1)

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1

University of Minnesota Medical Center

Minneapolis, Minnesota, United States, 55455