Current incidence, severity, and management of veno-occlusive disease/sinusoidal obstruction syndrome in adult allogeneic HSCT recipients: an EBMT Transplant Complications Working Party study

Author:

Ruutu TapaniORCID,Peczynski ChristopheORCID,Houhou Mohamed,Polge Emmanuelle,Mohty MohamadORCID,Kröger NicolausORCID,Moiseev IvanORCID,Penack OlafORCID,Salooja NinaORCID,Schoemans HélèneORCID,Duarte Rafael F.,Schroeder Thomas,Passweg Jakob,Wulf Gerald G.,Ganser Arnold,Sica Simona,Arat MutluORCID,Salmenniemi Urpu,Broers Annoek E. C.,Bourhis Jean Henri,Rambaldi Alessandro,Maertens JohanORCID,Halaburda Kazimierz,Zuckerman TsilaORCID,Labussière-Wallet Hélène,Basak GrzegorzORCID,Koenecke ChristianORCID,Perić ZinaidaORCID

Abstract

AbstractThe current incidence, diagnostic policy, management, and outcome of VOD/SOS at EBMT centers were studied. All centers that had performed allogeneic HSCTs in adult patients within one defined year were invited to the study. Seventy-one centers participated with a total of 2886 allogeneic transplantations and 93 cases of VOD/SOS in 2018. The cumulative incidence of VOD/SOS at day 21 was 1.8% and at day 100 2.4%. Of 67 cases with detailed data, 52 were classical and 15 (22%) late onset (>day 21). According to the EBMT criteria, 65/67 patients had at least two VOD/SOS risk factors. The severity grades were: mild 0, moderate 3, severe 29, very severe 35. Fifty-four patients were treated with defibrotide. VOD/SOS resolved in 58% of the patients, 3/3 with moderate, 22/28 with severe, and 12/33 with very severe grade (p < 0.001). By day 100, 57% of the patients were alive; 3/3 with moderate, 22/29 with severe, and 13/35 with very severe VOD/SOS (p = 0.002). In conclusion, the incidence of VOD/SOS was low. Severe and very severe grades dominated. Very severe grade predicted poor outcome compared to severe grade further supporting the concept of early diagnosis and treatment to avoid a dismal outcome.

Publisher

Springer Science and Business Media LLC

Subject

Transplantation,Hematology

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