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Hepatic veno-occlusive disease

MedGen UID:
5514
•Concept ID:
C0019156
•
Disease or Syndrome
Synonyms: Disease, Hepatic Veno-Occlusive; Hepatic Veno Occlusive Disease; Hepatic Veno-Occlusive Disease; Hepatic Veno-Occlusive Diseases; Sinusoidal Obstruction Syndrome; Syndrome, Sinusoidal Obstruction; Veno Occlusive Disease, Hepatic; Veno-Occlusive Disease, Hepatic
SNOMED CT: Veno-occlusive disease of the liver (65617004); Hepatic veno-occlusive disease (65617004); Hepatic sinusoidal obstruction syndrome (65617004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019514
Orphanet: ORPHA890

Definition

A rare vascular liver disease characterized by toxic injury to the hepatic sinusoidal capillaries that leads to obstruction of the small hepatic veins and sinusoids. Clinical manifestations include painful hepatomegaly, jaundice, and fluid retention that manifests by weight gain, edemas, and ascites. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHepatic veno-occlusive disease
Follow this link to review classifications for Hepatic veno-occlusive disease in Orphanet.

Professional guidelines

PubMed

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation.
Bonifazi F, Barbato F, Ravaioli F, Sessa M, Defrancesco I, Arpinati M, Cavo M, Colecchia A
Front Immunol 2020;11:489. Epub 2020 Apr 3 doi: 10.3389/fimmu.2020.00489. PMID: 32318059Free PMC Article
Modified diagnostic criteria, grading classification and newly elucidated pathophysiology of hepatic SOS/VOD after haematopoietic cell transplantation.
Cairo MS, Cooke KR, Lazarus HM, Chao N
Br J Haematol 2020 Sep;190(6):822-836. Epub 2020 Mar 4 doi: 10.1111/bjh.16557. PMID: 32133623Free PMC Article
Diagnosis, grading, and treatment recommendations for children, adolescents, and young adults with sinusoidal obstructive syndrome: an international expert position statement.
Mahadeo KM, Bajwa R, Abdel-Azim H, Lehmann LE, Duncan C, Zantek N, Vittorio J, Angelo J, McArthur J, Schadler K, Chan S, Tewari P, Khazal S, Auletta JJ, Choi SW, Shoberu B, Kalwak K, Harden A, Kebriaei P, Abe JI, Li S, Moffet JR, Abraham S, Tambaro FP, Kleinschmidt K, Richardson PG, Corbacioglu S; Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) Network and the Pediatric Diseases Working Party of the European Society for Blood and Marrow Transplantation
Lancet Haematol 2020 Jan;7(1):e61-e72. Epub 2019 Dec 7 doi: 10.1016/S2352-3026(19)30201-7. PMID: 31818728

Recent clinical studies

Etiology

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation.
Bonifazi F, Barbato F, Ravaioli F, Sessa M, Defrancesco I, Arpinati M, Cavo M, Colecchia A
Front Immunol 2020;11:489. Epub 2020 Apr 3 doi: 10.3389/fimmu.2020.00489. PMID: 32318059Free PMC Article
Modified diagnostic criteria, grading classification and newly elucidated pathophysiology of hepatic SOS/VOD after haematopoietic cell transplantation.
Cairo MS, Cooke KR, Lazarus HM, Chao N
Br J Haematol 2020 Sep;190(6):822-836. Epub 2020 Mar 4 doi: 10.1111/bjh.16557. PMID: 32133623Free PMC Article
Risk Factors for Development of and Progression of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.
Corbacioglu S, Jabbour EJ, Mohty M
Biol Blood Marrow Transplant 2019 Jul;25(7):1271-1280. Epub 2019 Feb 22 doi: 10.1016/j.bbmt.2019.02.018. PMID: 30797942
Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation.
Corbacioglu S, Carreras E, Ansari M, Balduzzi A, Cesaro S, Dalle JH, Dignan F, Gibson B, Guengoer T, Gruhn B, Lankester A, Locatelli F, Pagliuca A, Peters C, Richardson PG, Schulz AS, Sedlacek P, Stein J, Sykora KW, Toporski J, Trigoso E, Vetteranta K, Wachowiak J, Wallhult E, Wynn R, Yaniv I, Yesilipek A, Mohty M, Bader P
Bone Marrow Transplant 2018 Feb;53(2):138-145. Epub 2017 Jul 31 doi: 10.1038/bmt.2017.161. PMID: 28759025Free PMC Article
Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure.
Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, Arai S, Grupp SA, Guinan EC, Martin PL, Steinbach G, Krishnan A, Nemecek ER, Giralt S, Rodriguez T, Duerst R, Doyle J, Antin JH, Smith A, Lehmann L, Champlin R, Gillio A, Bajwa R, D'Agostino RB Sr, Massaro J, Warren D, Miloslavsky M, Hume RL, Iacobelli M, Nejadnik B, Hannah AL, Soiffer RJ
Blood 2016 Mar 31;127(13):1656-65. Epub 2016 Jan 29 doi: 10.1182/blood-2015-10-676924. PMID: 26825712Free PMC Article

Diagnosis

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation.
Bonifazi F, Barbato F, Ravaioli F, Sessa M, Defrancesco I, Arpinati M, Cavo M, Colecchia A
Front Immunol 2020;11:489. Epub 2020 Apr 3 doi: 10.3389/fimmu.2020.00489. PMID: 32318059Free PMC Article
Modified diagnostic criteria, grading classification and newly elucidated pathophysiology of hepatic SOS/VOD after haematopoietic cell transplantation.
Cairo MS, Cooke KR, Lazarus HM, Chao N
Br J Haematol 2020 Sep;190(6):822-836. Epub 2020 Mar 4 doi: 10.1111/bjh.16557. PMID: 32133623Free PMC Article
Acute Kidney Injury and CKD Associated with Hematopoietic Stem Cell Transplantation.
Renaghan AD, Jaimes EA, Malyszko J, Perazella MA, Sprangers B, Rosner MH
Clin J Am Soc Nephrol 2020 Feb 7;15(2):289-297. Epub 2019 Dec 13 doi: 10.2215/CJN.08580719. PMID: 31836598Free PMC Article
Risk Factors for Development of and Progression of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.
Corbacioglu S, Jabbour EJ, Mohty M
Biol Blood Marrow Transplant 2019 Jul;25(7):1271-1280. Epub 2019 Feb 22 doi: 10.1016/j.bbmt.2019.02.018. PMID: 30797942
Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure.
Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, Arai S, Grupp SA, Guinan EC, Martin PL, Steinbach G, Krishnan A, Nemecek ER, Giralt S, Rodriguez T, Duerst R, Doyle J, Antin JH, Smith A, Lehmann L, Champlin R, Gillio A, Bajwa R, D'Agostino RB Sr, Massaro J, Warren D, Miloslavsky M, Hume RL, Iacobelli M, Nejadnik B, Hannah AL, Soiffer RJ
Blood 2016 Mar 31;127(13):1656-65. Epub 2016 Jan 29 doi: 10.1182/blood-2015-10-676924. PMID: 26825712Free PMC Article

Therapy

Oxaliplatin-induced hepatic sinusoidal obstruction syndrome.
Zhu C, Ren X, Liu D, Zhang C
Toxicology 2021 Aug;460:152882. Epub 2021 Aug 2 doi: 10.1016/j.tox.2021.152882. PMID: 34352347
Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation.
Bonifazi F, Barbato F, Ravaioli F, Sessa M, Defrancesco I, Arpinati M, Cavo M, Colecchia A
Front Immunol 2020;11:489. Epub 2020 Apr 3 doi: 10.3389/fimmu.2020.00489. PMID: 32318059Free PMC Article
Modified diagnostic criteria, grading classification and newly elucidated pathophysiology of hepatic SOS/VOD after haematopoietic cell transplantation.
Cairo MS, Cooke KR, Lazarus HM, Chao N
Br J Haematol 2020 Sep;190(6):822-836. Epub 2020 Mar 4 doi: 10.1111/bjh.16557. PMID: 32133623Free PMC Article
Risk Factors for Development of and Progression of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.
Corbacioglu S, Jabbour EJ, Mohty M
Biol Blood Marrow Transplant 2019 Jul;25(7):1271-1280. Epub 2019 Feb 22 doi: 10.1016/j.bbmt.2019.02.018. PMID: 30797942
Sinusoidal obstruction syndrome.
Valla DC, Cazals-Hatem D
Clin Res Hepatol Gastroenterol 2016 Sep;40(4):378-85. Epub 2016 Mar 30 doi: 10.1016/j.clinre.2016.01.006. PMID: 27038846

Prognosis

Oxaliplatin-induced hepatic sinusoidal obstruction syndrome.
Zhu C, Ren X, Liu D, Zhang C
Toxicology 2021 Aug;460:152882. Epub 2021 Aug 2 doi: 10.1016/j.tox.2021.152882. PMID: 34352347
Acute Kidney Injury and CKD Associated with Hematopoietic Stem Cell Transplantation.
Renaghan AD, Jaimes EA, Malyszko J, Perazella MA, Sprangers B, Rosner MH
Clin J Am Soc Nephrol 2020 Feb 7;15(2):289-297. Epub 2019 Dec 13 doi: 10.2215/CJN.08580719. PMID: 31836598Free PMC Article
Inotuzumab ozogamicin versus standard of care in relapsed or refractory acute lymphoblastic leukemia: Final report and long-term survival follow-up from the randomized, phase 3 INO-VATE study.
Kantarjian HM, DeAngelo DJ, Stelljes M, Liedtke M, Stock W, Gökbuget N, O'Brien SM, Jabbour E, Wang T, Liang White J, Sleight B, Vandendries E, Advani AS
Cancer 2019 Jul 15;125(14):2474-2487. Epub 2019 Mar 28 doi: 10.1002/cncr.32116. PMID: 30920645Free PMC Article
Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation.
Corbacioglu S, Carreras E, Ansari M, Balduzzi A, Cesaro S, Dalle JH, Dignan F, Gibson B, Guengoer T, Gruhn B, Lankester A, Locatelli F, Pagliuca A, Peters C, Richardson PG, Schulz AS, Sedlacek P, Stein J, Sykora KW, Toporski J, Trigoso E, Vetteranta K, Wachowiak J, Wallhult E, Wynn R, Yaniv I, Yesilipek A, Mohty M, Bader P
Bone Marrow Transplant 2018 Feb;53(2):138-145. Epub 2017 Jul 31 doi: 10.1038/bmt.2017.161. PMID: 28759025Free PMC Article
Hepatic veno-occlusive disease.
Ribaud P, Gluckman E
Pediatr Transplant 1999;3 Suppl 1:41-4. doi: 10.1034/j.1399-3046.1999.00077.x. PMID: 10587970

Clinical prediction guides

Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a refined classification from the European society for blood and marrow transplantation (EBMT).
Mohty M, Malard F, Alaskar AS, Aljurf M, Arat M, Bader P, Baron F, Bazarbachi A, Blaise D, Brissot E, Ciceri F, Corbacioglu S, Dalle JH, Dignan F, Huynh A, Kenyon M, Nagler A, Pagliuca A, Perić Z, Richardson PG, Ruggeri A, Ruutu T, Yakoub-Agha I, Duarte RF, Carreras E
Bone Marrow Transplant 2023 Jul;58(7):749-754. Epub 2023 Apr 24 doi: 10.1038/s41409-023-01992-8. PMID: 37095231
Oxaliplatin-induced hepatic sinusoidal obstruction syndrome.
Zhu C, Ren X, Liu D, Zhang C
Toxicology 2021 Aug;460:152882. Epub 2021 Aug 2 doi: 10.1016/j.tox.2021.152882. PMID: 34352347
Inotuzumab ozogamicin versus standard of care in relapsed or refractory acute lymphoblastic leukemia: Final report and long-term survival follow-up from the randomized, phase 3 INO-VATE study.
Kantarjian HM, DeAngelo DJ, Stelljes M, Liedtke M, Stock W, Gökbuget N, O'Brien SM, Jabbour E, Wang T, Liang White J, Sleight B, Vandendries E, Advani AS
Cancer 2019 Jul 15;125(14):2474-2487. Epub 2019 Mar 28 doi: 10.1002/cncr.32116. PMID: 30920645Free PMC Article
Role of imaging techniques in liver veno-occlusive disease diagnosis: recent advances and literature review.
Ravaioli F, Colecchia A, Alemanni LV, Vestito A, Dajti E, Marasco G, Sessa M, Pession A, Bonifazi F, Festi D
Expert Rev Gastroenterol Hepatol 2019 May;13(5):463-484. Epub 2019 Mar 21 doi: 10.1080/17474124.2019.1588111. PMID: 30895833
Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure.
Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, Arai S, Grupp SA, Guinan EC, Martin PL, Steinbach G, Krishnan A, Nemecek ER, Giralt S, Rodriguez T, Duerst R, Doyle J, Antin JH, Smith A, Lehmann L, Champlin R, Gillio A, Bajwa R, D'Agostino RB Sr, Massaro J, Warren D, Miloslavsky M, Hume RL, Iacobelli M, Nejadnik B, Hannah AL, Soiffer RJ
Blood 2016 Mar 31;127(13):1656-65. Epub 2016 Jan 29 doi: 10.1182/blood-2015-10-676924. PMID: 26825712Free PMC Article

Recent systematic reviews

Efficacy and Safety of Recombinant Thrombomodulin for the Prophylaxis of Veno-Occlusive Complication in Allogeneiccit Hematopoietic Stem Cell Transplantation: A Systematic Review and Meta-Analysis.
Kashyap R, Anwer F, Iqbal MA, Khalid F, Khan A, Ali MA, Anwar MY, Chaudhary A, Jaan A
Hematol Oncol Stem Cell Ther 2023 Jan 17;16(2):93-101. doi: 10.1016/j.hemonc.2021.09.002. PMID: 34655527
A Systematic Review and Meta-Analysis of Studies of Defibrotide Prophylaxis for Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome.
Corbacioglu S, Topaloglu O, Aggarwal S
Clin Drug Investig 2022 Jun;42(6):465-476. Epub 2022 May 20 doi: 10.1007/s40261-022-01140-y. PMID: 35594010Free PMC Article
Veno-occlusive Disease in HSCT Patients: Consensus-based Recommendations for Risk Assessment, Diagnosis, and Management by the GITMO Group.
Bonifazi F, Sica S, Angeletti A, Marktel S, Prete A, Iori AP, Olivari D, Rossetti G, Bertaina A, Botti S, Busca A, Carella AM, Cerretti R, Gargiulo G, Grassi A, Gualandi F, Irrera G, Milone G, Risitano AM, Santarone S, Vassallo E, Zecca M, Ciceri F, Pomponio G
Transplantation 2021 Apr 1;105(4):686-694. doi: 10.1097/TP.0000000000003569. PMID: 33273315
Interventions for prophylaxis of hepatic veno-occlusive disease in people undergoing haematopoietic stem cell transplantation.
Cheuk DK, Chiang AK, Ha SY, Chan GC
Cochrane Database Syst Rev 2015 May 27;(5):CD009311. doi: 10.1002/14651858.CD009311.pub2. PMID: 26017019
Defibrotide for the prevention of hepatic veno-occlusive disease after hematopoietic stem cell transplantation: a systematic review.
Zhang L, Wang Y, Huang H
Clin Transplant 2012 Jul-Aug;26(4):511-9. Epub 2012 Mar 20 doi: 10.1111/j.1399-0012.2012.01604.x. PMID: 22428954

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