Case Analysis Of Severe Acquired Aplastic Anemia In Children

Objective:The clinical characterISTics of severe aplastic anemia(SAA)in children were analyzed retrospectively.To analyze the efficacy of combined immunosuppressive therapy(IST)with antithymocyte globulin(ATG)combined with cyclosporine A(CsA)and CsA alone.To investigate the effect of allogeneic hematopoietic stem cell transplantation on severe aplastic anemia in children.Methods:A total of 45 cases of children with SAA and extremely severe aplastic anemia(vSAA)admitted to the pediatric blood group of our hospital from January 2014 to December 2018 were retrospectively analyzed,and the general conditions such as gender,age,course of disease and clinical manifestations at the time of onset were summarized,and the characterISTics of laboratory examination at the initial diagnosis were analyzed.Different treatments were applied to evaluate the therapeutic effect.The diagnostic and therapeutic criteria of the selected children were in line with the recommendations for the diagnosis and treatment of children acquired aplastic anemia(2014 edition).Results:1.Children with acquired SAA were more likely to be of pre-school age and school-age.School-age children were the most common group.There were 30 boys and 15 girls with a 2:1 ratio,with a median age of 8 years(1-13),35 cases of severe aplastic anemia(SAA),and 10 cases of extremely severe aplastic anemia(vSAA).Most of the children had typical symptoms such as pale complexion,fatigue,skin and mucous membrane bleeding spots,ecchymosis and fever.2.2% of the children had superficial cervical lymph node enlargement,all the children had no hepatosplenomegaly,35.6% of the children had primary infection,all the children had a three-line reduction,bone marrow biopsy showed a different degree of reduction in bone marrow hyperplasia,megacyte occasionally or absent,lymphocytes and fat cells were easy to see,non-hematopoietic tissue proliferation.2.All the 21 pediatric patients who were treated with IST were first treated with IST,among which 10 cases were basically cured,5 cases were in complete remission,and 2 cases were significantly improved.The total effective rate was 81%.Four cases were ineffective,and one of the very severe cases was allergic reaction.Seven children were treated with CsA,of which 1 was basically cured,2 were relieved,1 was significantly improved,3 were ineffective,and the total effective rate was 57.1%.Follow-up ranged from 3 months to 5 years.3.14 cases were treated with peripheral blood hematopoietic stem cell transplantation(PBSCT),and all three lines were successfully implanted.Four children developed acute graft-versus-host disease(aGVHD)after transplantation,one developed chronic graft-versus-host disease after transplantation,five developed hemorrhagic cystitis,and eight developed infection after pretreatment or transplantation,all of which are disease-free.4.3 the children died,all of which were in critical condition at admission.One child with SAA developed cerebral hemorrhage after diagnosis,and died in vain.Two vSAA children died in the course of combined anti-infection and blood transfusion.Conclutions:1.For severe aplastic anemia in children,the basic cure rate of hematopoietic stem cell transplantation in different treatment schemes is significantly better than that of CsA or IST alone.2.For children without suitable hematopoietic stem cell transplantation donors,IST is still the first-line treatment for severe aplastic anemia in children.3.Children with SAA and vSAA are in a critical condition,which requires early diagnosis and appropriate treatment options.