RESULTS OF ALLOGENEIC BONE-MARROW TRANSPLANTATION IN PATIENTS WITH LEUKOCYTE ADHESION DEFICIENCY

We have retrospectively analyzed the outcome of bone marrow transplant ation (BMT) in 14 patients with leukocyte adhesion deficiency (LAD) pe rformed in two centers between 1981 and 1993. Five patients received B MT from HLA-identical donors, Nine received T-depleted marrow from two HLA antigen- or haplotype-incompatible parents. Conditioning regimen consisted of chemotherapy exclusively in 13 patients and associated wi th total body irradiation (TBI) in one patient, In five cases, failure of engraftment occurred as a result of either insufficient myeloablat ion (n = 3) or, possibly, graft rejection in two cases of moderate phe notype of LAD, The second conditioning regimen consisted of TBI and ch emotherapy with the use of anti-lymphocyte function-associated antigen 1 (LFA-1) and anti-CD2 monoclonal antibodies for patients with the mo derate phenotype of LAD. These patients were successfully retransplant ed. Eight patients developed acute graft-versus-host disease (GVHD), C hronic GVHD occurred in five cases. GVHD led to the death of three pat ients, Ten patients are alive and well 12 months to 12 years after BMT . Chimerism is full in six of these patients and mixed but stable in f our with variable proportion of donor leukocytes. One patient with les s than 15% donor leukocytes has mild gingivitis, while the others are well, Sequelae from BMT are limited in two cases to growth retardation caused by TBI. Success of BMT in cases of LAD including seven of nine recipients of HLA nonidentical marrow indicates that this procedure c an be proposed as a curative approach to LAD regardless of an availabl e HLA-identical donor. Great care should be taken in GVHD prophylaxis and treatment, (C) 1995 by The American Society of Hematology.