Clinical Study of Post-Transplantation Lymphoproliferative Diseases in Children with Acquired Aplastic Anemia After Allogeneic Hematopoietic Stem Cell Transplantation

Abstract

Objective To explore the epidemiology,clinical characteristics,diagnosis,treatment and prognosis of post-transplantation lymphoproliferative diseases (PTLD) in children with acquired aplastic anemia (AAA) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods From July 2002 to May 2012,a total of 71 children who underwent allo-HSCT with AAA at the Institute of Shanghai Children's Medical Center were included in this study.All of these children's clinical data were analyzed.The conditioning regimen included fludarabine,cyclophosphamide and rabbit-antithymocyte globulin with or without total body radiotherapy,and graft-versus-host disease prophylaxis mainly consisted of cyclosporine and methotrexate.For these patients who were monitored by Epstein-Barr virus (EBV)-DNA copies in peripheral blood mononuclear cells after allo-HSCT,a preemptive treatment with anti-CD20 monoclonal antibody rituximab was given when EBV-DNA copies rose persistently.Once PTLD was diagnosed,reduction or withdrawal of immunosuppression was administered.Results The incidence of PTLD for children with AAA after allo-HSCT was 4.2％ (3/71).All the PTLD patients were presented with persistent fever with no reaction to any antibiotics,progressive antiadoncus and lymphadenectasis.Two cases of the PTLD patients were monitored by EBV-DNA copies after allo-HSCT,and both had persistent high level of EBV-DNA copies before the diagnosis of PTLD,also after the first course of rituximab.After therapies,two of the three PTLD patients were cured and alive till now; however,another one had no effect to the therapy and died 34 days after the diagnosis of PTLD.Conclusions PTLD is a rare and potentially fatal complication for children with AAA after allo-HSCT.Dynamic monitoring of EBV-DNA copies could have certain guiding on the early diagnosis and treatment for PTLD.The preemptive treatment with rituximab could reduce the incidence and mortality of PTLD. Key words: post-transplantation lymphoproliferative diseases; acquired aplastic anemia; allogeneic hematopoietic stem cell transplantation; children; Epstein-Barr virus; rituximab