Matched Unrelated Hematopoietic Stem Cell Transplantation Using Selected CD34 super(+) Cells in Fanconi's Anemia: Experience of One Center

In order to prevent Graft versus Host Disease (GvHD) in Matched Unrelated Donor Hematopoietic Stem Cell Transplantation (MUD-HSCT) in Fanconi's Anemia (FA), we report the results of 15 children transplanted by CD34 super(+) positive cells selected by immunomagnetic method isolex/isolex 300i (Baxter Healthcare Corp. USA) in the period between April 1996 and September 1999. Methods: The median total nucleated cells infused 3x10 super(8)E kg super(-1) (0, 65- 3, 95), CD34 cells 2.12x10 super(6)E kg super(-1) (0.7-5.8), CD3 cells 2.56x10 super(6)E kg super(-1) (0.001-0.5). Median follow-up is 131 days (34-3022) till 06/2005. All patients achieved engraftment except 2 and another 2 rejected early post transplant. Acute GVHD > grade II and extensive chronic GVHD occurred in 4 cases and one case, respectively. Ten/Fifteen patients died. Transplant related mortality in 8 patients (graft failure n = 4, fungal infection n = 3 and GVHD n = 1), 2ry leukemia and relapse of initial disease in one patient, respectively. Five cases are alive and well 6 years at least post transplant. Positive selection of CD34 cells minimizes GVHD in Fanconi's anemia patients and gives acceptable survival. However, graft failure remains the main obstacle.