Standard diagnosis and treatment in childhood aplastic anemia
XIE Xiao-tian(Department of Pediatric Hematology and Oncology,Shanghai Tongji Hospital Affiliated to Shanghai Tongji University,Shanghai 200065,China)
Aplastic anemia(AA)is one of the severe hematopoietic diseases in children. The diagnosis and treatment of AA are much difficult in clinic and the prognosis of severe AA(SAA)is very poor. Because China is one of the areas with high incidence of AA in the world,More attention should be paid to laboratory and clinical research of childhood AA in the country. It has been confirmed that immune-mediated pathogenesis of AA and the abnormal T lymphocytes' inhibiting bone marrow hematopoietic stem cells play important roles in AA pathogenesis. Although successful allogeneic hematopoietic stem cell transplantation(allo-HSCT)could cure AA,allo-HSCT is restricted in use, especially in our country,due to limited donors,high cost and high medical risk,etc. Immunosuppressive therapy(IS)based on immune-mediated pathogenesis has achieved very good outcome in the treatment of AA in recent years,and IS has been confirmed as the best alternative treatment for SAA and refractory AA when no suitable donor for allo-HSCT is available. Recent progress in AA in the world,the standard of diagnosis and classification,main points of differentiate diagnosis,principle and methods of treatment,and the standard of treatment effects of AA have been discussed.