T&B Depletion Non Malignant
Graft Versus Host Disease
About this trial
This is an interventional prevention trial for Graft Versus Host Disease focused on measuring indication for HSCT, matched related donor, MRD, Matched Unrelated Donor, MUD
Eligibility Criteria
Inclusion Criteria:
- non malignant haematological and inherited metabolic disorders benefiting from an allogeneic HSCT conditioned with a myeloablative regimen
- Availability of a matched related donor (MRD) or Matched Unrelated Donor (MUD)
- Lansky or Karnofsky Index ≥ 60
- Inherited metabolic disorders: DQ ≥ 70 (+ MRI Loes score ≤ 9 for adrenoleukodystrophy)
- Adequate cardiac, renal, hepatic and pulmonary functions as evidenced by:
- Serum creatinine ≤ 1.5 × upper limit of normal (ULN)
- Heart shortening fraction (left-ventricle) > 28 % or LVEF > 55%
- Serum bilirubin ≤ 1.5 × ULN (except for Wolman disease),
- AST and ALT ≤ 2.5 × ULN (except for thalassemic syndromes and Wolman disease)
- Pulmonary function: if cooperative: FEV1 and FVC on pulmonary function testing > 60 %; if non cooperative: pulse oximetry > 95 % in room air
- Availability of autologous back up marrow (> 2 x 108 TNC+ cells/kg or > 2 x 106 CD34+ cells/kg) for MUD
- Adequate contraception in female patients of child-bearing potential
- Signed informed consent
Exclusion Criteria:
- Any malignancy
- Liver cirrhosis evidenced on liver histology (performed in suspicious cases or in case of Wolman disease)
- HIV- positivity
- Clinically significant pleural effusion or ascites
- Pregnancy or lactation
- Known hypersensitivity to trial drugs
- Participation in another experimental drug trial in the 2 months preceding enrollment
- Non-cooperative behaviour or non-compliance
- Previous HSCT
Sites / Locations
- University of Cagliari
- San Raffaele Scientific Institute
- University of Milano-Bicocca San Gerardo Hospital
- Bambino Gesù Hospital and Research InstituteRecruiting
Arms of the Study
Arm 1
Arm 2
Arm 3
Arm 4
Experimental
Sham Comparator
Experimental
Sham Comparator
MRD-Regimen&Polyclonal antibody
MRD-Regimen
MUD-Regimen & Rituximab
MUD-Regimen
Patients MRD will be randomized to receive Treosulfan iv at a dose of 14 g/m² within 120 minutes on day -7, - 6, -5 + Fludarabine iv at a dose of 30 mg/ m² within 30 minutes on day -7, -6, -5,-4,-3 after treosulfan + Thiotepa iv at a dose of 8 mg/kg on day - 3 divided into 2 infusions at 12 hrs intervals + Cyclosporine A iv at a dose of 3 mg/kg/day starting from day -1 and a dose adjustment will be done to obtain plasma levels of 150-250 ng/mL + Methotrexate iv at a dose of 15 mg/m2 on day +1, at a dose of 10 mg/m2 on day + 3 and + 6 & ATG-Fresenius S® iv at a dose of 5 mg/kg within 8 hours on day -4,-3,-2
Patients receiving stem cell transplantation from a matched related donors (MRD) will be randomized to receive only Treosulfan iv at a dose of 14 g/m² within 120 minutes on day -7, - 6, -5 (total dose of 42 g/m²) + Fludarabine iv at a dose of 30 mg/ m² within 30 minutes on day -7, -6, -5,-4,-3 (total dose of 150 mg/m²) after treosulfan + Thiotepa iv at a dose of 8 mg/kg on day - 3 divided into 2 infusions at 12 hrs intervals (total dose of 8 mg/kg)+ Cyclosporine A iv at a starting dose of 3 mg/kg/day starting from day -1 and a dose adjustment will be done to obtain plasma levels of 150-250 ng/mL (In the absence of GvHD CSA will be tapered after day + 180 and stopped at 9-12 months) + Methotrexate iv at a dose of 15 mg/m2 on day +1, at a dose of 10 mg/m2 on day + 3 and + 6
Patients MUD will be randomized to receive Treosulfan iv at a dose of 14 g/m² within 120 minutes on day -7, - 6, -5 + Fludarabine iv at a dose of 30 mg/ m² within 30 minutes on day -7, -6, -5,-4,-3 after treosulfan + Thiotepa iv at a dose of 8 mg/kg on day - 3 divided into 2 infusions at 12 hrs intervals + Cyclosporine A iv at a dose of 3 mg/kg/day starting from day -1 and a dose adjustment will be done to obtain plasma levels of 150-250 ng/mL + Methotrexate iv at a dose of 15 mg/m2 on day +1, at a dose of 10 mg/m2 on day + 3 and + 6 and at a dose of 10 mg/m2 on day +11 + ATG-Fresenius S ® iv at a dose of 5 mg/kg within 8 hours on day -4,-3,-2 & Rituximab in a single infusion of 200 mg/m2 on day -1
Patients MUD will be randomized to receive only Treosulfan iv at a dose of 14 g/m² within 120 minutes on day -7, - 6, -5 + Fludarabine iv at a dose of 30 mg/ m² within 30 minutes on day -7, -6, -5,-4,-3 after treosulfan + Thiotepa iv at a dose of 8 mg/kg on day - 3 divided into 2 infusions at 12 hrs intervals + Cyclosporine A iv at a dose of 3 mg/kg/day starting from day -1 and a dose adjustment will be done to obtain plasma levels of 150-250 ng/mL + Methotrexate iv at a dose of 15 mg/m2 on day +1, at a dose of 10 mg/m2 on day + 3 and + 6 and at a dose of 10 mg/m2 on day +11 + ATG-Fresenius S ® iv at a dose of 5 mg/kg within 8 hours on day -4,-3,-2