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An international survey to better understand the current incidence, severity, and management of VOD/SOS

Bone Marrow Transplantation volume 60, pages 28–31 (2025)Cite this article

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Abstract

This international questionnaire survey aimed to explore the current incidence, diagnostic policies, management, and outcomes of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) among healthcare providers involved in the management of these patients. A questionnaire was e-mailed to practitioners with an interest in allogeneic hematopoietic cell transplantation (allo-HCT). Of the respondents, 144 of 215 (67.0%) felt that early detection or diagnosis of VOD/SOS was difficult. Regarding diagnostic criteria, 142 (66.1%) already declared using the 2023 EBMT refined criteria. Most respondents (163/215, 75.8%) found these recent refined EBMT criteria useful for diagnosis, and 193 (89.8%) found the severity criteria easy to use. The major risk factors identified for VOD/SOS were a second allo-HCT (41.4%), pre-existing liver disease (54.9%), and prior use of antibody-drug conjugates (49.8%). Preferences for starting VOD/SOS treatment varied, with 61 (28.4%) preferring initiating therapy at a mild stage, and 122 (56.7%) preferring the moderate stage. In summary, this survey provided valuable insight into the challenges and opportunities of the identification and management of VOD/SOS. By improving current knowledge and increasing collaboration among healthcare professionals, early detection, management, and clinical outcomes for patients with this potentially serious complication can also be improved.

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Fig. 1: The unmodifiable VOD/SOS risk factors for adults from the refined EBMT criteria: percentage of participants who consider them important.
Fig. 2: The modifiable VOD/SOS risk factors for adults from the refined EBMT criteria: Percentage of participants who consider them important.

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Data availability

Data sharing is available through the IACH office (info@clinical-hematology.org) and Dr Myriam Labopin (myriam.labopin@upmc.fr).

References

  1. Vythoulkas D, Tsirigotis P, Griniezaki M, Konstantellos I, Lazana I. Endothelial dysfunction syndromes after allogeneic stem cell transplantation. Cancers (Basel). 2023;15:680.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Corbacioglu S, Jabbour EJ, Mohty M. Risk factors for development of and progression of hepatic veno-occlusive disease/sinusoidal obstruction syndrome. Biol Blood Marrow Transplant. 2019;25:1271–80.

    Article  PubMed  Google Scholar 

  3. Marcoux C, Saliba RM, Wallis W, Khazal S, Ragoonanan D, Rondon G, et al. Incidence and risk factors of early onset VOD/SOS differ in younger vs older adults after stem cell transplantation. Blood Adv. 2024;8:1128–36.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Corbacioglu S, Carreras E, Ansari M, Balduzzi A, Cesaro S, Dalle JH, et al. 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. Bone Marrow Transplant. 2018;53:138–45.

    Article  CAS  PubMed  Google Scholar 

  5. Corbacioglu S, Topaloglu O, Aggarwal S. A systematic review and meta-analysis of studies of defibrotide prophylaxis for veno-occlusive disease/sinusoidal obstruction syndrome. Clin Drug Investig. 2022;42:465–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Jones RJ, Lee KS, Beschorner WE, Vogel VG, Grochow LB, Braine HG, et al. Venoocclusive disease of the liver following bone marrow transplantation. Transplantation. 1987;44:778–83.

    Article  CAS  PubMed  Google Scholar 

  7. Mohty M, Malard F, Alaskar AS, Aljurf M, Arat M, Bader P, et al. 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). Bone Marrow Transplant. 2023;58:749–54.

    Article  PubMed  Google Scholar 

  8. Ichikawa H, Yakushijin K, Kurata K, Tsuji T, Takemoto N, Joyce M, et al. Utility of the refined EBMT diagnostic and severity criteria 2023 for sinusoidal obstruction syndrome/veno-occlusive disease. Bone Marrow Transplant. 2024;59:518–25.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Cairo MS, Cooke KR, Lazarus HM, Chao N. Modified diagnostic criteria, grading classification and newly elucidated pathophysiology of hepatic SOS/VOD after haematopoietic cell transplantation. Br J Haematol. 2020;190:822–36.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Dignan FL, Wynn RF, Hadzic N, Karani J, Quaglia A, Pagliuca A, et al. BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation. Br J Haematol. 2013;163:444–57.

    Article  CAS  PubMed  Google Scholar 

  11. Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, et al. Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure. Blood. 2016;127:1656–65.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Nauffal M, Kim HT, Richardson PG, Soiffer RJ, Antin JH, Cutler C, et al. Defibrotide: real-world management of veno-occlusive disease/sinusoidal obstructive syndrome after stem cell transplant. Blood Adv. 2022;6:181–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Bearman SI, Anderson GL, Mori M, Hinds MS, Shulman HM, McDonald GB. Venoocclusive disease of the liver: development of a model for predicting fatal outcome after marrow transplantation. J Clin Oncol. 1993;11:1729–36.

    Article  CAS  PubMed  Google Scholar 

  14. Mohty M, Malard F, Abecassis M, Aerts E, Alaskar AS, Aljurf M, et al. Revised diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a new classification from the European Society for Blood and Marrow Transplantation. Bone Marrow Transplant. 2016;51:906–12.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Bonifazi F, Barbato F, Ravaioli F, Sessa M, Defrancesco I, Arpinati M, et al. Diagnosis and treatment of VOD/SOS after allogeneic hematopoietic stem cell transplantation. Front Immunol. 2020;11:489.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Wallhult E, Kenyon M, Liptrott S, Mank A, Ní Chonghaile M, Babic A, et al. Management of veno-occlusive disease: the multidisciplinary approach to care. Eur J Haematol. 2017;98:322–9.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Sorbonne Université, Centre de Recherche Saint-Antoine INSERM UMRs938, Service d’Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Paris, France

    Marion Larue, Myriam Labopin, Eolia Brissot, Florent Malard & Mohamad Mohty

  2. King Abdullah International Medical Research Center, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia

    Ahmed S. Alaskar

  3. King Faisal Specialist Hospital and Research Center Riyadh, Riyadh, Saudi Arabia

    Mahmoud Aljurf

  4. F Sisli Florence Nightingale Hospital, HSCT Unit, Istanbul, Turkey

    Mutlu Arat

  5. CHU and University of Liège, Liège, Belgium

    Frederic Baron

  6. BMT program, department of internal medicine, American University of Beirut, Beirut, Lebanon

    Ali Bazarbachi

  7. San Raffaele Scientific Institute, Hematology and Bone marrow Transplantation Unit, Milan, Italy

    Fabio Ciceri & Annalisa Ruggeri

  8. Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Regensburg, Regensburg, Germany

    Selim Corbacioglu

  9. Department of clinical haematology, Manchester University NHS Foundation Trust, Manchester, UK

    Fiona L. Dignan

  10. Department of Haematology, King’s College Hospital, London, UK

    Michelle Kenyon & Antonio Pagliuca

  11. Hematology and Bone Marrow Transplantation, Chaim Sheba Medical center, Tel-Hashomer, Israel

    Arnon Nagler

  12. CHU de Lille, université de Lille, Infinite, Inserm U1286, 59000, Lille, France

    Ibrahim Yakoub-Agha

  13. Bone Marrow Transplantation Center, Zhejiang University School of Medicine, Hangzhou, China

    Yishan Ye

  14. Department of Hematology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain

    Rafael F. Duarte

  15. Clinical Research Institute and Comprehensive Cancer Center, Helsinki University Hospital, Helsinki, Finland

    Tapani Ruutu

  16. Josep Carreras Foundation & Leukemia Research Institute, Barcelona, Spain

    Enric Carreras

  17. Department of Hematology, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, Zagreb, Croatia

    Zinaida Peric

  18. EBMT ALWP office, Hôpital Saint-Antoine, Paris, France

    Mohamad Mohty

Authors
  1. Marion Larue
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  2. Myriam Labopin
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  4. Ahmed S. Alaskar
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  15. Antonio Pagliuca
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  19. Rafael F. Duarte
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  21. Enric Carreras
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  22. Zinaida Peric
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  23. Mohamad Mohty
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Contributions

MM and ML conceived and designed the study. All authors collected the data. MM and ML analyzed the data. MM and ML wrote the manuscript. All authors critically revised and approved the final manuscript. MM is the guarantors of the study, had full access to all the data, and took responsibility for the integrity of the data and the accuracy of the data analysis.

Corresponding author

Correspondence to Marion Larue.

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Competing interests

Ahmed Alaskar: Received honoraria/consultancy fees: Novartis, Abbvie, Janssen, Takeda, Kyowa Kirin, Gilead, Roche, Sanofi. Participated in company sponsored speakers bureau: Janssen, Kyowa Kirin, Sanofi. Michelle Kenyon: Received honoraria/consultancy fees: Jazz Pharmaceuticals, Sanofi, Roche, Mallinkrodt, Vertex. Speakers bureau: Jazz Pharmaceuticals, Sanofi, Pfizer.

Ethics approval

The IACH steering committee (www.iach.org) approved the conduct of this survey according to standard professional practices.

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Larue, M., Labopin, M., Brissot, E. et al. An international survey to better understand the current incidence, severity, and management of VOD/SOS. Bone Marrow Transplant 60, 28–31 (2025). https://doi.org/10.1038/s41409-024-02434-9

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