Abstract
A 4-year-old boy with early relapse of B-cell precursor acute lymphoblastic leukemia of the bone marrow and central nervous system underwent HLA-identical (8/8 serotypes) allogeneic peripheral blood stem cell transplantation from his mother. He developed dry skin on day 102 after transplant and was diagnosed as having chronic graft-versus-host disease. Treatment with prednisolone was initiated. However, he developed sclerodermatous skin changes 1 year after the transplant and scoliosis that progressed with his growth. Nine years after the transplant, the patient developed dyspnea with progressive restrictive lung disease due to restriction of thoracic respiratory motion and hypoventilation resulting from obstruction of the right main bronchus due to progression of scoliosis. Steroid treatment was ineffective, and imatinib was initiated to target his skin lesions. His body surface area was 0.80 m2, and imatinib was initiated at a dose of 100 mg/day for the first 2 weeks, after which the dose was increased to 200 mg/day. Six months after initiating imatinib, improvements were seen in his Rodnan score, which decreased from 36 to 27; in SpO2, which increased from 85% to 95%; and in PCO2, which decreased from 70 mmHg to 50 mmHg. The only adverse reaction was mild diarrhea.
