Radiologic resolution of malignant infantile osteopetrosis skeletal changes following hematopoietic stem cell transplantation.

摘要: Introduction Hematopoietic stem cell transplantation (HSCT) is the only known curative treatment of malignant infantile osteopetrosis (MIOP). In this study, short-term serial bone surveys were used to assess radiologic evolution skeletal changes after HSCT in MIOP. Materials and Methods Baseline whole-body survey was performed all patients. successful 14 patients (11 with full chimerism, three mixed chimerism) whom follow-up carried out at 6 12 months HSCT. Results Normal corticomedullary differentiation evident five (P = 0.06) (P < 0.005) months, respectively. Abnormal endobone appearance long bones, present 11 participants baseline exam, disappeared eight (P = 0.008) (P = 0.001) 6-month follow-up, rachitic significantly (P < 0.01) bones; however, they ribs (P = 0.50). No patient had rickets or bones months. Conclusion We observed considerable resolution MIOP either chimerism. Rachitic attenuated differentiation, first resolve. We propose using single plain x-ray demonstrate response HSCT. Pediatr Blood Cancer 2015;62:1645–1649. © 2015 Wiley Periodicals, Inc.