Trial Outcomes & Findings for Related Hematopoietic Stem Cell Transplantation (HSCT) for Genetic Diseases of Blood Cells (NCT NCT02512679)

NCT ID: NCT02512679

Last Updated: 2017-02-27

Results Overview

Absolute Neutrophil Count (ANC) =/\>500;(recovery of white cell count - self sustain platelet above 20,000 per cubic milimeter (20K) - evaluation by Chimerism Study (STR or FISH) at day +30

Recruitment status

TERMINATED

Study phase

PHASE2

Target enrollment

20 participants

Primary outcome timeframe

30 days

Results posted on

2017-02-27

Participant Flow

Patients for bone marrow transplantation who met the eligibility criteria of diagnosis and clinical status and had histocompatible sibling donor. These patients were recruited at Children's Hospital Los Angeles and Lucille Packard Children's Hospital at Stanford University. Patients were recruited between 2006 and 2013.

This is a sequential dose de-escalation of Cyclophosphamide with first at 105 mg/kg total dose compared to standard 200mg/kg total dose (Arm 1). Prior to enrollment patients had to meet all eligibility criteria for allogeneic transplantation. Study closed after 1st level because all patients engrafted and none had toxicity related to transplant.

Participant milestones

Participant milestones
Measure
Cyclophosphamide Dose Level 1
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Cyclophosphamide Dose Level 2
De-escalation of Cyclophosphamide given by Intravenous (IV) at a total dose of 70 mg/kg, to be divided into two doses of one 35 mg/kg dose per day, for 2 days Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 75 mg/kg, to be divided into two doses of one 35 mg/kg dose per day, for 2 days. After ten patients the de-escalation will begin if the stopping rule is not met.
Overall Study
STARTED
20
0
Overall Study
COMPLETED
20
0
Overall Study
NOT COMPLETED
0
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Related Hematopoietic Stem Cell Transplantation (HSCT) for Genetic Diseases of Blood Cells

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Cyclophosphamide Dose Level 1
n=20 Participants
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Age, Categorical
<=18 years
19 Participants
n=99 Participants
Age, Categorical
Between 18 and 65 years
1 Participants
n=99 Participants
Age, Categorical
>=65 years
0 Participants
n=99 Participants
Age, Continuous
2.3 years
n=99 Participants
Gender
Female
13 Participants
n=99 Participants
Gender
Male
7 Participants
n=99 Participants
Region of Enrollment
United States
20 participants
n=99 Participants

PRIMARY outcome

Timeframe: 30 days

Population: Subject enrolled and received Dose Level 1 of Cyclophosphamide and engrafted with Absolute Neutrophil Count (ANC) =/\>500;(recovery of white cell count - self sustain platelet above 20k - evaluation by Chimerism Study (STR or FISH) at day +30.

Absolute Neutrophil Count (ANC) =/\>500;(recovery of white cell count - self sustain platelet above 20,000 per cubic milimeter (20K) - evaluation by Chimerism Study (STR or FISH) at day +30

Outcome measures

Outcome measures
Measure
Cyclophosphamide Dose Level 1
n=20 Participants
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Number of Participants With Neutrophil Engraftment (=/>500 Cells/uL) and Platelet Engraftment (>20K Cell/uL) at 30 Days
20 participants

PRIMARY outcome

Timeframe: 1 year

Population: All subjects who received dose level 1 of Cyclophosphamide did not experience re-occurrence of disease.

assess rate of disease recurrence ("late relapse") due to autologous recovery of recipient hematopoiesis at one year post-HSCT.

Outcome measures

Outcome measures
Measure
Cyclophosphamide Dose Level 1
n=20 Participants
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Number of Participants With Disease Recurrence at 1 Year Post-transplant
0 participants

PRIMARY outcome

Timeframe: 1 year

Population: Subjects who received dose level 1 of Cyclophosphamide did not experience veno-occlusive disease, organ failure or severe mucositis.

Assessment of conditioning regimen related toxicity was evaluated and documented with daily assessment during hospitalization and post-transplant follow-up up to one year. None of the subjects developed VOD necessitating any therapeutic intervention, severe mucositis, or toxicity of the Kidney, Liver or Gastrointestinal.

Outcome measures

Outcome measures
Measure
Cyclophosphamide Dose Level 1
n=20 Participants
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Number of Participants Who Developed Severe Mucositis, Veno-occlusive Disease (VOD), Toxicity of the Kidney, Liver, or Gastrointestinal (GI) Tract up to 1 Year Post-transplant
Veno-Occlusive Disease
0 participants
Number of Participants Who Developed Severe Mucositis, Veno-occlusive Disease (VOD), Toxicity of the Kidney, Liver, or Gastrointestinal (GI) Tract up to 1 Year Post-transplant
Viral Infection
8 participants
Number of Participants Who Developed Severe Mucositis, Veno-occlusive Disease (VOD), Toxicity of the Kidney, Liver, or Gastrointestinal (GI) Tract up to 1 Year Post-transplant
Toxicity of Kidney, Liver, or Gastrointestinal
0 participants
Number of Participants Who Developed Severe Mucositis, Veno-occlusive Disease (VOD), Toxicity of the Kidney, Liver, or Gastrointestinal (GI) Tract up to 1 Year Post-transplant
Severe Mucositis
0 participants

SECONDARY outcome

Timeframe: 1 yr

Population: Subjects who received dose level 1 of Cyclophosphamide were revaluated for graft-versus-host disease (GVHD) post transplantation.

Clinical evaluation on a daily basis during hospitalization and at each post transplant clinical visit, up to one year, to determine incidence of acute and chronic graft-versus-host disease using Glucksberg grading scale. Acute graft-versus-host disease (aGVHD) develops within the first three months after transplantation and appears as a skin rash, often accompanied by hyperbilirubenemia, abnormal liver enzymes and gastrointestinal symptoms, as diarrhea, nausea and vomiting. Level of aGVHD is graded from 1-4. Chronic GVHD, typically a late complication of Blood and Marrow Transplantation (BMT) characterized by skin changes, sometimes sclerotic changes, with joint contractures, liver function abnormality, gastrointestinal symptoms and sometime other organ involvement such as eyes, lungs, and obliterative bronchiolitis (OB). Chronic GVHD is graded as absent, limited, or extensive.

Outcome measures

Outcome measures
Measure
Cyclophosphamide Dose Level 1
n=20 Participants
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Number of Participants Who Developed Graft-Versus-Host-Disease (GVHD) as Determined by the Glucksberg Scale
Acute GVHD (Grade 1-2)
10 participants
Number of Participants Who Developed Graft-Versus-Host-Disease (GVHD) as Determined by the Glucksberg Scale
Acute GVHD (Grade 3-4)
0 participants
Number of Participants Who Developed Graft-Versus-Host-Disease (GVHD) as Determined by the Glucksberg Scale
Chronic GVHD
0 participants

SECONDARY outcome

Timeframe: 1 yr

Population: Subjects receiving dose level 1 of Cyclophosphamide event free survival post transplant at 100 days was 95% and 90% 1 year.

Evaluation for engraftment, correction of the disease, transplant related complications and event-free survival and overall survival of the subjects post-transplant was undertaken by standard measures and evaluation of disease with disease-specific testing.

Outcome measures

Outcome measures
Measure
Cyclophosphamide Dose Level 1
n=20 Participants
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Number of Participants Who Were Disease Progression-Free and Death-Free at 1 Year Post-transplant
100 Day Event Free Survival Post Transplant
19 participants
Number of Participants Who Were Disease Progression-Free and Death-Free at 1 Year Post-transplant
One Year Event Free Survival Post Transplant
18 participants
Number of Participants Who Were Disease Progression-Free and Death-Free at 1 Year Post-transplant
Disease Progression-Free
20 participants

Adverse Events

Cyclophosphamide Dose Level 1

Serious events: 2 serious events
Other events: 7 other events
Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure
Cyclophosphamide Dose Level 1
n=20 participants at risk
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Respiratory, thoracic and mediastinal disorders
Death due to pre-exisiting mucormycosis
5.0%
1/20 • Number of events 1 • Adverse event data was collected throughout the course of the study up to 2 years post transplantation. Subjects were evaluated for post transplant complications related to conditioning, such as VOD, GVHD, infections and engraftment failure.
Respiratory, thoracic and mediastinal disorders
Death due to interstitial pneumonia
5.0%
1/20 • Number of events 1 • Adverse event data was collected throughout the course of the study up to 2 years post transplantation. Subjects were evaluated for post transplant complications related to conditioning, such as VOD, GVHD, infections and engraftment failure.

Other adverse events

Other adverse events
Measure
Cyclophosphamide Dose Level 1
n=20 participants at risk
Cyclophosphamide given by Intravenous (IV) at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. Drug to be given in combination of Busulfan, Campath and Fludarabine Cyclophosphamide Dose Level 1: given by IV at a total dose of 105 mg/kg, to be divided into three doses of one 35 mg/kg dose per day, for 3 days on the first level. After ten patients the de-escalation will begin if the stopping rule is not met.
Blood and lymphatic system disorders
Cytomegalovirus (CMV) infection
25.0%
5/20 • Number of events 5 • Adverse event data was collected throughout the course of the study up to 2 years post transplantation. Subjects were evaluated for post transplant complications related to conditioning, such as VOD, GVHD, infections and engraftment failure.
Blood and lymphatic system disorders
Epstein-Barr Virus (EBV)
5.0%
1/20 • Number of events 1 • Adverse event data was collected throughout the course of the study up to 2 years post transplantation. Subjects were evaluated for post transplant complications related to conditioning, such as VOD, GVHD, infections and engraftment failure.
Blood and lymphatic system disorders
Adenovirus
5.0%
1/20 • Number of events 1 • Adverse event data was collected throughout the course of the study up to 2 years post transplantation. Subjects were evaluated for post transplant complications related to conditioning, such as VOD, GVHD, infections and engraftment failure.

Additional Information

Neena Kapoor, M.D.

Children's Hospital Los Angeles

Phone: 343-361-2434

Results disclosure agreements

  • Principal investigator is a sponsor employee
  • Publication restrictions are in place