TBI Dose De-escalation for Fanconi Anemia

NCT ID: NCT00352976

Last Updated: 2021-11-24

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE2/PHASE3

Total Enrollment

83 participants

Study Classification

INTERVENTIONAL

Study Start Date

2006-05-18

Study Completion Date

2020-10-09

Brief Summary

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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

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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 cGy) with thymic shielding; it will be given six days before the stem cells are given (day -6). 2) Day -5 through Day -2, subjects will receive a chemotherapy regimen of Fludarabine and Cyclophosphamide via central line (i.e. Hickman or Broviac). Starting Day -3, patients will receive sirolimus therapy with a taper commencing on day +180 and also mycophenolate mofetil (MMF) through day +30 or for 7 days after engraftment, whichever day is later, if no acute graft-versus-host disease (GVHD). 4) If the subject is receiving bone marrow or "peripheral" stem cells (cells collected from the donor's arm via a cell separator), on the day of transplantation, the stem cells taken from the donor will be put into a machine which will separate the lymphocytes (the cells that cause graft-versus-host disease \[GVHD\]) from the stem cells. If the subject is receiving an umbilical cord blood, the lymphocytes will not be removed because the risk of GVHD is not as high. Otherwise all patients will receive the same treatment. The stem cells are given as an infusion into the subject's existing catheter over 1-2 hours on day 0.5. On the day after transplant (day +1) subjects will be given G-CSF to stimulate the growth of the transplanted cells. 6. While receiving treatment and until the subject's blood counts recover he/she will have daily blood tests, and several bone marrow biopsies and aspirates. After recovery, subjects will be seen once a month for a health assessment and blood tests until at least 3 months after the cells have been infused. Additional blood tests or assessments may be done as medically indicated.

Conditions

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Fanconi Anemia

Keywords

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Bone Marrow transplant stem cell transplant cord blood transplant total body irradiation thymic shielding

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Treatment with TBI

Patients treated with total body irradiation, Fludarabine, Cyclophosphamide, Bone Marrow Transplantation, Mycophenolate Mofetil, and Sirolimus.

Group Type EXPERIMENTAL

Cyclophosphamide

Intervention Type DRUG

Day -5 through Day -2, subjects will receive chemotherapy of Cyclophosphamide via central line (i.e. Hickman or Broviac),10 mg/kg intravenously (IV)

Fludarabine

Intervention Type DRUG

Day -5 through Day -2 prior to transplant; subjects will receive chemotherapy of Fludarabine via central line (i.e. Hickman or Broviac),35 mg/m\^2 intravenous (IV)

Total Body Irradiation

Intervention Type PROCEDURE

total body irradiation (300 cGy) with thymic shielding will be given six days before the stem cells are given (day -6). Thymic shielding is done by placing a piece of lead on the chest during the irradiation treatment so that the irradiation beams do not go to the thymus.

Bone Marrow Transplantation

Intervention Type PROCEDURE

A target of 5 \* 10\^6/kg and a minimum of 4 \* 10\^6 CD34+ cell/kg recipient weight will be collected by apheresis and used for transplant. In most cases this dose will be recovered in a single apheresis; however, a second or rarely third apheresis performed on the following days may be required to achieve the minimum dose.

Mycophenolate Mofetil

Intervention Type DRUG

Patients will receive MMF therapy beginning on day -3 through day +30 or for 7 days after engraftment, whichever day is later, if no acute graft-versus-host disease (GVHD). Engraftment is defined as 1st day of 3 consecutive days of absolute neutrophil count \[ANC\] \> 0.5 \* 10\^9/L. MMF will be given at a dose of 15 mg/kg/dose every 8 hours by mouth(to a maximum dose of 1 gram).

Sirolimus

Intervention Type DRUG

Sirolimus will be administered starting at day -3 with 8mg-12mg mg oral loading dose followed by single dose 4 mg/day with a target serum concentration of 3 to 12 mg/mL by high-performance liquid chromatography (HPLC). Levels are to be monitored 3 times/week in the first 2 weeks, weekly until day +60, and as clinically indicated until day +100 post-transplantation. In the absence of acute GVHD sirolimus may be tapered starting at day +100 and eliminated by day +180 post-transplantation.

Interventions

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Cyclophosphamide

Day -5 through Day -2, subjects will receive chemotherapy of Cyclophosphamide via central line (i.e. Hickman or Broviac),10 mg/kg intravenously (IV)

Intervention Type DRUG

Fludarabine

Day -5 through Day -2 prior to transplant; subjects will receive chemotherapy of Fludarabine via central line (i.e. Hickman or Broviac),35 mg/m\^2 intravenous (IV)

Intervention Type DRUG

Total Body Irradiation

total body irradiation (300 cGy) with thymic shielding will be given six days before the stem cells are given (day -6). Thymic shielding is done by placing a piece of lead on the chest during the irradiation treatment so that the irradiation beams do not go to the thymus.

Intervention Type PROCEDURE

Bone Marrow Transplantation

A target of 5 \* 10\^6/kg and a minimum of 4 \* 10\^6 CD34+ cell/kg recipient weight will be collected by apheresis and used for transplant. In most cases this dose will be recovered in a single apheresis; however, a second or rarely third apheresis performed on the following days may be required to achieve the minimum dose.

Intervention Type PROCEDURE

Mycophenolate Mofetil

Patients will receive MMF therapy beginning on day -3 through day +30 or for 7 days after engraftment, whichever day is later, if no acute graft-versus-host disease (GVHD). Engraftment is defined as 1st day of 3 consecutive days of absolute neutrophil count \[ANC\] \> 0.5 \* 10\^9/L. MMF will be given at a dose of 15 mg/kg/dose every 8 hours by mouth(to a maximum dose of 1 gram).

Intervention Type DRUG

Sirolimus

Sirolimus will be administered starting at day -3 with 8mg-12mg mg oral loading dose followed by single dose 4 mg/day with a target serum concentration of 3 to 12 mg/mL by high-performance liquid chromatography (HPLC). Levels are to be monitored 3 times/week in the first 2 weeks, weekly until day +60, and as clinically indicated until day +100 post-transplantation. In the absence of acute GVHD sirolimus may be tapered starting at day +100 and eliminated by day +180 post-transplantation.

Intervention Type DRUG

Other Intervention Names

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cytoxan fludara Radiation Therapy, Therapeutic radiation Stem Cell transplantation MMF Rapamycin

Eligibility Criteria

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Inclusion Criteria

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 Criteria

* 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).
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Masonic Cancer Center, University of Minnesota

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Margaret L MacMillan, M.D.

Role: PRINCIPAL_INVESTIGATOR

University of Minnesota Medical Center

Locations

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University of Minnesota Medical Center

Minneapolis, Minnesota, United States

Site Status

Countries

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United States

References

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MacMillan ML, DeFor TE, Young JA, Dusenbery KE, Blazar BR, Slungaard A, Zierhut H, Weisdorf DJ, Wagner JE. Alternative donor hematopoietic cell transplantation for Fanconi anemia. Blood. 2015 Jun 11;125(24):3798-804. doi: 10.1182/blood-2015-02-626002. Epub 2015 Mar 30.

Reference Type BACKGROUND
PMID: 25824692 (View on PubMed)

Provided Documents

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Document Type: Study Protocol and Statistical Analysis Plan

View Document

Related Links

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https://www.ncbi.nlm.nih.gov/pubmed/25824692

Trial 4 listed in the article corresponds to this clinical trial.

Other Identifiers

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0605M85788

Identifier Type: OTHER

Identifier Source: secondary_id

MT2006-05

Identifier Type: -

Identifier Source: org_study_id