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

M Mohty, F Malard, M Abecassis, E Aerts, A S Alaskar, M Aljurf, M Arat, P Bader, F Baron, A Bazarbachi, D Blaise, F Ciceri, S Corbacioglu, J-H Dalle, F Dignan, T Fukuda, A Huynh, T Masszi, M Michallet, A Nagler, M NiChonghaile, S Okamoto, A Pagliuca, C Peters, F B Petersen, P G Richardson, T Ruutu, B N Savani, E Wallhult, I Yakoub-Agha, R F Duarte, E Carreras, M Mohty, F Malard, M Abecassis, E Aerts, A S Alaskar, M Aljurf, M Arat, P Bader, F Baron, A Bazarbachi, D Blaise, F Ciceri, S Corbacioglu, J-H Dalle, F Dignan, T Fukuda, A Huynh, T Masszi, M Michallet, A Nagler, M NiChonghaile, S Okamoto, A Pagliuca, C Peters, F B Petersen, P G Richardson, T Ruutu, B N Savani, E Wallhult, I Yakoub-Agha, R F Duarte, E Carreras

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. Although SOS/VOD progressively resolves within a few weeks in most patients, the most severe forms result in multi-organ dysfunction and are associated with a high mortality rate (>80%). Therefore, careful attention must be paid to allow an early detection of SOS/VOD, particularly as drugs have now proven to be effective and licensed for its treatment. Unfortunately, current criteria lack sensitivity and specificity, making early identification and severity assessment of SOS/VOD difficult. The aim of this work is to propose a new definition for diagnosis, and a severity-grading system for SOS/VOD in adult patients, on behalf of the European Society for Blood and Marrow Transplantation.

Conflict of interest statement

All authors received honoraria and/or research support from JAZZ Pharmaceuticals whose product is discussed in this manuscript. JAZZ pharmaceuticals provided an unrestricted educational grant for support for the current study, but did not participate to the discussions, conduct of the work, data/results analyses or manuscript writing or reviewing. All authors designed the manuscript, analyzed the literature, wrote and commented on the manuscript. All authors approved submission of the manuscript for publication purposes.

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