title: [Early diagnosis and comprehensive treatments of post-transplantation lymphoproliferative disorder after pediatric liver transplantation].
abstract: OBJECTIVE
  To summarize the clinical characteristics, early diagnosis, comprehensive treatment and prognosis of 6 cases of children with post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation.
  
  
  METHODS
  Data of 6 cases with PTLD seen between January 2011 and December 2013 were retrospectively analyzed. The anti-rejection drug dose adjustments, the effect of rituximab, antiviral therapy and comprehensive treatment program after surgery were explored.
  
  
  RESULTS
  (1) The diagnosis of PTLD was confirmed by histologic findings. Six cases of PTLD including 3 males and 3 females were diagnosed as congenital biliary atresia and underwent split liver transplantation. The occurrence rate of PTLD was 2.9%. (2) The median time to the development of PTLD was less than 6 months. The initial symptom of PTLD in all patients was fever and clinical manifestations of PTLD were non-specific, depending on the involving organs. Five cases of PTLD developed gastrointestinal symptoms, including diarrhea, abdominal pain, and abdominal distension. One case developed respiratory symptoms, including cough and tachypnea. Three cases had lymph node involvement. In 2 cases pathophysiology involved polymorphic lymphocyte proliferation and in 4 cases B lymphocyte proliferation. (3) Two cases died, in whom EBV DNA was not detected and were diagnosed as PTLD by surgical pathology before death. Four survived cases had high EBV-DNA load and then were diagnosed as PTLD by biopsy pathology. (4) Of the 6 cases of PTLD, 2 cases died and 4 cases survived. The overall mortality was 33%. The dead cases were only treated with laparotomy because of intestinal obstruction or perforation and the survived cases were treated with tacrolimus at reduced doses or discontinuation and rituximab. In 2 cases antiviral therapy (acyclovir) was continued, including 1 cases of intestinal obstruction treated with surgical repair. All the survived patients were followed up for 4 months to 1 year and no evidence has been found.
  
  
  CONCLUSIONS
  EBV infection is the high risk factor for PTLD after liver transplantation. Close clinical surveillance of EBV DNA for pediatric liver transplantation was important for the early diagnosis of PTLD. Reducing doses of immunosuppressive agents and rituximab is the initial therapy for PTLD. A reduction in the dose of tacrolimus is suggested. Operation therapy can also play a role in the management of local complications.
authors.affiliation: Department of Pediatrics, Shanghai Children's Medical Center, Shanghai Jiaotong University Medical College, Shanghai 200127, China.
authors.forename: Zhaohui
authors.initials: Z
authors.lastname: Deng
authors.affiliation: Email: jiangl_rong@aliyun.com.
authors.forename: Lirong
authors.initials: L
authors.lastname: Jiang
authors.forename: Tao
authors.initials: T
authors.lastname: Zhou
authors.forename: Conghuan
authors.initials: C
authors.lastname: Shen
authors.forename: Qimin
authors.initials: Q
authors.lastname: Chen
authors.forename: Qiang
authors.initials: Q
authors.lastname: Xia
mesh_terms: D000998:Antiviral Agents / Q000008:administration & dosage*; D001656:Biliary Atresia / Q000628:therapy; D004279:DNA, Viral / Q000032:analysis; D004359:Drug Therapy, Combination; D042241:Early Diagnosis; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000628:therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007223:Infant; D016031:Liver Transplantation / Q000009:adverse effects*; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy*; D008297:Male; D010372:Pediatrics; D011183:Postoperative Complications; D012189:Retrospective Studies; D015996:Survival Rate; D016559:Tacrolimus / Q000008:administration & dosage
chemical_list: D000998:Antiviral Agents; D004279:DNA, Viral; D007166:Immunosuppressive Agents; D016559:Tacrolimus