Table 1 Transplant-related data of patients
From: Farber disease: clinical presentation, pathogenesis and a new approach to treatment
Parameter | Patients | |||
|---|---|---|---|---|
Patient number and gender | #1, female | #2, male | #3, female | #4, female |
Patient origin | Muenster | Muenster | Muenster | Hamburg |
Age at SCT | 3 years | 3 years | 2 years | 21 years |
Preparative regimen | Bu/Cy | Bu/Cy/ATG | Bu/Cy | Bu/Cy/ATG |
Stem cell source and dose (no T-cell depletion) | BM (MRD), 11.3 × 106 CD34 | PBSC (MUD), 25 × 106 CD34 | BM (MRD), 5.6 × 106 CD34 | BM (MRD), 4.2 × 106 CD34 |
GvHD prophylaxis | CsA, MTX | CsA, MTX | CsA, MTX | CsA, MTX |
Neutrophil engraftment | day +14 | day +12 | day +18 | day +14 |
Acute/chronic GvHD | grade II of gut/none | grade I of gut/none | grade II of skin/none | none |
Therapy of acute/chronic GvHD | steroids/none | steroids/none | steroids + tacrolimus/none | none |
Infections | Clostridium difficile enteritis, bacteremia with Staphylococci | CMV reactivation | CMV colitis | none |
Toxicities | CsA-neurotoxicity | None | CsA-neurotoxicity | suspected VOD |
Current donor cell chimerism | 91% | 95% | 90% | 99% |