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)

Mohamad Mohty; Florent Malard; Ahmed S Alaskar; Mahmoud Aljurf; Mutlu Arat; Peter Bader; Frederic Baron; Ali Bazarbachi; Didier Blaise; Eolia Brissot; Fabio Ciceri; Selim Corbacioglu; Jean-Hugues Dalle; Fiona Dignan; Anne Huynh; Michelle Kenyon; Arnon Nagler; Antonio Pagliuca; Zinaida Perić; Paul G Richardson; Annalisa Ruggeri; Tapani Ruutu; Ibrahim Yakoub-Agha; Rafael F Duarte; Enric Carreras

doi:10.1038/s41409-023-01992-8

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 Jul;58(7):749-754. doi: 10.1038/s41409-023-01992-8. Epub 2023 Apr 24.

Authors

Mohamad Mohty^#¹, Florent Malard^#², Ahmed S Alaskar^#³, Mahmoud Aljurf^#⁴, Mutlu Arat^#⁵, Peter Bader^#⁶, Frederic Baron^#⁷, Ali Bazarbachi^#⁸, Didier Blaise^#⁹, Eolia Brissot^#², Fabio Ciceri^#¹⁰, Selim Corbacioglu^#¹¹, Jean-Hugues Dalle^#¹², Fiona Dignan^#¹³, Anne Huynh^#¹⁴, Michelle Kenyon^#¹⁵, Arnon Nagler^#¹⁶, Antonio Pagliuca^#¹⁵, Zinaida Perić^#¹⁷, Paul G Richardson^#¹⁸, Annalisa Ruggeri^#¹⁰, Tapani Ruutu^#¹⁹, Ibrahim Yakoub-Agha^#²⁰, Rafael F Duarte^#²¹, Enric Carreras^#²²

Affiliations

¹ Hematology Department, Hôpital Saint-Antoine, and Université Pierre & Marie Curie, Paris, France. mohamad.mohty@inserm.fr.
² Hematology Department, Hôpital Saint-Antoine, and Université Pierre & Marie Curie, Paris, France.
³ King Abdullah International Medical Research Center/King Saud Bin Abdulaziz University for Health Sciences, Dept. of Oncology, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
⁴ King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
⁵ Sisli Florence Nightingale Hospital, HSCT Unit, Istanbul, Turkey.
⁶ Division for Stem Cell Transplantation and Immunology, Department for Children and Adolescents, University Hospital, Goethe University, Frankfurt/Main, Germany.
⁷ Department of Hematology, University of Liege, Liege, Belgium.
⁸ Department of Internal Medicine, American University of Beirut, Beirut, Lebanon.
⁹ Aix-Marseille Univ, MSC Lab, Institut Paoli-Calmettes, Department of Hematology, Marseille, France.
¹⁰ San Raffaele Scientific Institute, Hematology and Bone marrow Transplantation Unit, Milan, Italy.
¹¹ Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Regensburg, Regensburg, Germany.
¹² Department of Hematology and Immunology, Hospital Robert Debre, Paris 7-Paris Diderot University, Paris, France.
¹³ Department of Clinical Haematology, Manchester University NHS Foundation Trust, Manchester, UK.
¹⁴ Hematology Department, Institut Universitaire du Cancer Toulouse - Oncopole, Toulouse, France.
¹⁵ Department of Haematology, King's College Hospital, London, UK.
¹⁶ Hematology and Bone Marrow Transplantation, Chaim Sheba Medical center, Tel-Hashomer, Israel.
¹⁷ Department of Hematology, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, Zagreb, Croatia.
¹⁸ Division of Hematology, Mayo Clinic, Rochester, MN, USA.
¹⁹ Clinical Research Institute, Helsinki University Hospital, Helsinki, Finland.
²⁰ CHU de Lille, Univ Lille, INSERM U1286, Infinite, 59000, Lille, France.
²¹ Department of Hematology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.
²² Josep Carreras Foundation & Leukemia Research Institute, (Hospital Clínic/Barcelona University Campus), Barcelona, Spain.

^# Contributed equally.

PMID: 37095231
DOI: 10.1038/s41409-023-01992-8

Abstract

Sinusoidal obstruction syndrome, also known as veno-occlusive disease (SOS/VOD), is a potentially life-threatening complication that can develop after hematopoietic cell transplantation (HCT). A new definition for diagnosis, and a severity grading system for SOS/VOD in adult patients, was reported a few years ago on behalf of the European Society for Blood and Marrow Transplantation (EBMT). The aim of this work is to update knowledge regarding diagnosis and severity assessment of SOS/VOD in adult patients, and also its pathophysiology and treatment. In particular, we now propose to refine the previous classification and distinguish probable, clinical and proven SOS/VOD at diagnosis. We also provide an accurate definition of multiorgan dysfunction (MOD) for SOS/VOD severity grading based on Sequential Organ Failure Assessment (SOFA) score.

Publication types

Review

MeSH terms

Adult
Bone Marrow
Hematopoietic Stem Cell Transplantation* / adverse effects
Hepatic Veno-Occlusive Disease* / diagnosis
Hepatic Veno-Occlusive Disease* / drug therapy
Hepatic Veno-Occlusive Disease* / etiology
Humans
Syndrome
Vascular Diseases*