title: [Delayed Hepatic Veno-Occlusive Disease after Haploidentical Hematopoietic Stem Cell Transplantation:A Report of Six Cases].
abstract: OBJECTIVE
  To evaluate the morbidity, risk factors, clinical characterisitics, treatments and prognosis of delayed hepatic veno-occlusive disease(HVOD) after haploidentical hematopoietic stem cell transplantation (hi-HSCT).
  
  
  METHODS
  The clinical data of 208 patients undergoing hi-HSCT were retrospectively analyzed.
  
  
  RESULTS
  Six patients were diagnosed with delayed VOD, among them 4 patients were moderate VOD and 2 patients were severe VOD. The incidence of VOD after hi-HSCT was 2.88%, the median onset time was 44.5(30-57) days after transplant, 2 patients died of multiple organ failure (MOF) due to rapid progress of disease. With intravenous administration of defibrotide, 4 patients displayed encouraging response, but 2 patients died of hepatic acute graft-versus-host disease (aGVHD), 1 had bone marrow relapse and the other one was cured.
  
  
  CONCLUSIONS
  Norethindrone is one of the high risk factors, while sex, age and disease status are irrelevant to the occurrence of VOD. Unfractionated heparin (UH) can effectively decrease the morbidity. Pretransplant hepatic function reserve, high dose preconditioning regimens and pharmacotherapy may result in delayed VOD onset. The delayed VOD has the same clinical features and treatment-response as early VOD, but a poorer prognosis is usually observed. A larger amount of samples (patients) is needed to research the relationship of the delayed VOD with hi-HSCT. Defibrotide can effectively increase the survival rate of VOD patients.
authors.affiliation: Department of Hematology, Clinical College of Chinese Air Force, Anhui Medical University, Beijing 100142.
authors.forename: Hui-Zhu
authors.initials: HZ
authors.lastname: Kang
authors.affiliation: Department of Hematology, General Hospital of Chinese Air Force, Beijing 100142, China.
authors.forename: Xiao-Li
authors.initials: XL
authors.lastname: Zheng
authors.affiliation: Department of Hematology, General Hospital of Chinese Air Force, Beijing 100142, China.
authors.forename: Zhi-Dong
authors.initials: ZD
authors.lastname: Wang
authors.affiliation: Department of Hematology, General Hospital of Chinese Air Force, Beijing 100142, China.
authors.forename: Dong-Mei
authors.initials: DM
authors.lastname: Han
authors.affiliation: Department of Hematology, General Hospital of Chinese Air Force, Beijing 100142, China.
authors.forename: Li
authors.initials: L
authors.lastname: Ding
authors.affiliation: Department of Hematology, Clinical College of Chinese Air Force, Anhui Medical University, Beijing 100142.E-mail:wanghengxiang123@aliyun.com.
authors.forename: Heng-Xiang
authors.initials: HX
authors.lastname: Wang
mesh_terms: D006086:Graft vs Host Disease; D006238:Haploidy; D018380:Hematopoietic Stem Cell Transplantation; D006493:Heparin; D006504:Hepatic Veno-Occlusive Disease; D006801:Humans; D015994:Incidence; D011089:Polydeoxyribonucleotides; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D011089:Polydeoxyribonucleotides; C036901:defibrotide; D006493:Heparin