title: [Clinical characteristics of hepatic veno-occlusive disease in 6 children with hematologic neoplasm treated with 6-thioguanine].
abstract: OBJECTIVE
  To improve the treatment of drug related childhood hepatic veno-occlusive disease (HVOD), clinical characteristics of 6 children with hematologic neoplasm from 2 hospitals of China Children's Leukemia Group (CCLG) treated with 6-thioguanine (6-TG) complicated with HVOD were analyzed.
  
  
  METHODS
  All the drug related HVOD patients were treated with CCLG acute lymphoblastic leukemia (ALL)-2008 protocol. They were from Children's Hospital of Fudan University and Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College from April 2008 to April 2009. The diagnosis was made according to the modified Seattle criteria and Baltimore criteria, including 2 or 3 of the following clinical features: hepatomegaly and upper right abdominal pain, jaundice (bilirubin ≥ 35 µmol/L), ascites or confirmed by pathology. The 6 HVOD patients' clinical manifestations, laboratory finding, imageologic and pathologic data were collected and analyzed.
  
  
  RESULTS
  Of the 6 patients, 2 were males and 4 females. Mean age of the 6 patients was 3.89 years (range from 3 years 1 month to 4 years 11 months). The original disease was acute lymphoblastic leukemia. HVOD occurred during chemotherapy protocols of CAM (CTX + Ara-C + 6-TG) or maintenance period (MTX + 6-TG). Most of 6 HVOD patients presented with pain in liver area, hepatomegaly on imaging, elevated aminotransferase and bilirubin (often ≥ 35 µmol/L), hydroperitonia was common, one with pleural fluid, illegible hepatic veins. All the patients recovered after being treated with hepatoprotective, jaundice-relieving and supportive therapeutics, some patients were treated with low molecular weight heparin. The prognoses were good.
  
  
  CONCLUSIONS
  HVOD was a serious complication of chemotherapy with 6-TG. Hepatoprotective and jaundice-relieving and low molecular weight heparin could improve the prognosis.
authors.affiliation: Department of Hematology, Children's Hospital of Fudan University, Shanghai 201102, China.
authors.forename: Hong-sheng
authors.initials: HS
authors.lastname: Wang
authors.forename: Yi-jin
authors.initials: YJ
authors.lastname: Gao
authors.forename: Jun
authors.initials: J
authors.lastname: Li
authors.forename: Feng-juan
authors.initials: FJ
authors.lastname: Lu
authors.forename: Hui
authors.initials: H
authors.lastname: Miao
authors.forename: Xiao-wen
authors.initials: XW
authors.lastname: Qian
authors.forename: Xiao-fan
authors.initials: XF
authors.lastname: Zhu
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002675:Child, Preschool; D005260:Female; D006504:Hepatic Veno-Occlusive Disease / Q000188:drug therapy*; D006801:Humans; D007938:Leukemia / Q000628:therapy; D008297:Male; D013866:Thioguanine / Q000627:therapeutic use*
chemical_list: D000970:Antineoplastic Agents; D013866:Thioguanine