[en] 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.
Disciplines :
Hematology
Author, co-author :
Larue, Marion ; 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@hotmail.fr
Labopin, Myriam ; Sorbonne Université, Centre de Recherche Saint-Antoine INSERM UMRs938, Service d'Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Paris, France
Brissot, Eolia ; Sorbonne Université, Centre de Recherche Saint-Antoine INSERM UMRs938, Service d'Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Paris, France
Alaskar, Ahmed S; King Abdullah International Medical Research Center, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia
Aljurf, Mahmoud; King Faisal Specialist Hospital and Research Center Riyadh, Riyadh, Saudi Arabia
Arat, Mutlu ; F Sisli Florence Nightingale Hospital, HSCT Unit, Istanbul, Turkey
Baron, Frédéric ; Université de Liège - ULiège > Département des sciences cliniques
Bazarbachi, Ali ; BMT program, department of internal medicine, American University of Beirut, Beirut, Lebanon
Ciceri, Fabio ; San Raffaele Scientific Institute, Hematology and Bone marrow Transplantation Unit, Milan, Italy
Corbacioglu, Selim ; Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Regensburg, Regensburg, Germany
Dignan, Fiona L; Department of clinical haematology, Manchester University NHS Foundation Trust, Manchester, UK
Kenyon, Michelle ; Department of Haematology, King's College Hospital, London, UK
Malard, Florent; Sorbonne Université, Centre de Recherche Saint-Antoine INSERM UMRs938, Service d'Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Paris, France
Nagler, Arnon ; Hematology and Bone Marrow Transplantation, Chaim Sheba Medical center, Tel-Hashomer, Israel
Pagliuca, Antonio ; Department of Haematology, King's College Hospital, London, UK
Ruggeri, Annalisa ; San Raffaele Scientific Institute, Hematology and Bone marrow Transplantation Unit, Milan, Italy
Yakoub-Agha, Ibrahim ; CHU de Lille, université de Lille, Infinite, Inserm U1286, 59000, Lille, France
Ye, Yishan ; Bone Marrow Transplantation Center, Zhejiang University School of Medicine, Hangzhou, China
Duarte, Rafael F; Department of Hematology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
Ruutu, Tapani ; Clinical Research Institute and Comprehensive Cancer Center, Helsinki University Hospital, Helsinki, Finland
Carreras, Enric ; Josep Carreras Foundation & Leukemia Research Institute, Barcelona, Spain
Peric, Zinaida; Department of Hematology, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, Zagreb, Croatia
Mohty, Mohamad ; Sorbonne Université, Centre de Recherche Saint-Antoine INSERM UMRs938, Service d'Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Paris, France ; EBMT ALWP office, Hôpital Saint-Antoine, Paris, France
D. Vythoulkas P. Tsirigotis M. Griniezaki I. Konstantellos I. Lazana Endothelial dysfunction syndromes after allogeneic stem cell transplantation Cancers (Basel). 15 680 1:CAS:528:DC%2BB3sXjtlars74%3D 10.3390/cancers15030680 36765638 9913851
S. Corbacioglu E.J. Jabbour M. Mohty Risk factors for development of and progression of hepatic veno-occlusive disease/sinusoidal obstruction syndrome Biol Blood Marrow Transplant. 25 1271 80 10.1016/j.bbmt.2019.02.018 30797942
C. Marcoux R.M. Saliba W. Wallis S. Khazal D. Ragoonanan G. Rondon et al. Incidence and risk factors of early onset VOD/SOS differ in younger vs older adults after stem cell transplantation Blood Adv. 8 1128 36 1:CAS:528:DC%2BB2cXltVGjsLk%3D 10.1182/bloodadvances.2023011233 38266155 10909702
S. Corbacioglu E. Carreras M. Ansari A. Balduzzi S. Cesaro J.H. Dalle 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. 53 138 45 1:STN:280:DC%2BC1cfis1Ogug%3D%3D 10.1038/bmt.2017.161 28759025
S. Corbacioglu O. Topaloglu S. Aggarwal A systematic review and meta-analysis of studies of defibrotide prophylaxis for veno-occlusive disease/sinusoidal obstruction syndrome Clin Drug Investig. 42 465 76 1:CAS:528:DC%2BB38XhvVaku7bI 10.1007/s40261-022-01140-y 35594010 9188533
R.J. Jones K.S. Lee W.E. Beschorner V.G. Vogel L.B. Grochow H.G. Braine et al. Venoocclusive disease of the liver following bone marrow transplantation Transplantation. 44 778 83 1:STN:280:DyaL1c7gtVyhsw%3D%3D 10.1097/00007890-198712000-00011 3321587
M. Mohty F. Malard A.S. Alaskar M. Aljurf M. Arat P. Bader 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. 58 749 54 10.1038/s41409-023-01992-8 37095231
H. Ichikawa K. Yakushijin K. Kurata T. Tsuji N. Takemoto M. Joyce et al. Utility of the refined EBMT diagnostic and severity criteria 2023 for sinusoidal obstruction syndrome/veno-occlusive disease Bone Marrow Transplant. 59 518 25 1:CAS:528:DC%2BB2cXitlOqsLs%3D 10.1038/s41409-024-02215-4 38287083 10994840
M.S. Cairo K.R. Cooke H.M. Lazarus N. Chao Modified diagnostic criteria, grading classification and newly elucidated pathophysiology of hepatic SOS/VOD after haematopoietic cell transplantation Br J Haematol. 190 822 36 10.1111/bjh.16557 32133623 7483983
F.L. Dignan R.F. Wynn N. Hadzic J. Karani A. Quaglia A. Pagliuca et al. BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation Br J Haematol. 163 444 57 1:CAS:528:DC%2BC3sXhs12mtLvL 10.1111/bjh.12558 24102514
P.G. Richardson M.L. Riches N.A. Kernan J.A. Brochstein S. Mineishi A.M. Termuhlen et al. Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure Blood. 127 1656 65 1:CAS:528:DC%2BC28Xhs1ehurrN 10.1182/blood-2015-10-676924 26825712 4817309
M. Nauffal H.T. Kim P.G. Richardson R.J. Soiffer J.H. Antin C. Cutler et al. Defibrotide: real-world management of veno-occlusive disease/sinusoidal obstructive syndrome after stem cell transplant Blood Adv. 6 181 8 1:CAS:528:DC%2BB38XhtVOrsbnN 10.1182/bloodadvances.2021005410 34666352 8753224
S.I. Bearman G.L. Anderson M. Mori M.S. Hinds H.M. Shulman G.B. McDonald Venoocclusive disease of the liver: development of a model for predicting fatal outcome after marrow transplantation J Clin Oncol. 11 1729 36 1:STN:280:DyaK3szlvF2hsA%3D%3D 10.1200/JCO.1993.11.9.1729 8355040
M. Mohty F. Malard M. Abecassis E. Aerts A.S. Alaskar M. Aljurf 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. 51 906 12 1:CAS:528:DC%2BC28XotFahtbY%3D 10.1038/bmt.2016.130 27183098 4935979
F. Bonifazi F. Barbato F. Ravaioli M. Sessa I. Defrancesco M. Arpinati et al. Diagnosis and treatment of VOD/SOS after allogeneic hematopoietic stem cell transplantation Front Immunol. 11 489 1:CAS:528:DC%2BB3cXitVWnu77L 10.3389/fimmu.2020.00489 32318059 7147118
E. Wallhult M. Kenyon S. Liptrott A. Mank M. Ní Chonghaile A. Babic et al. Management of veno-occlusive disease: the multidisciplinary approach to care Eur J Haematol. 98 322 9 10.1111/ejh.12840 27992075
