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      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

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          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.

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          Most cited references68

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          American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference: definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis.

          (1992)
          To define the terms "sepsis" and "organ failure" in a precise manner. Review of the medical literature and the use of expert testimony at a consensus conference. American College of Chest Physicians (ACCP) headquarters in Northbrook, IL. Leadership members of ACCP/Society of Critical Care Medicine (SCCM). An ACCP/SCCM Consensus Conference was held in August of 1991 with the goal of agreeing on a set of definitions that could be applied to patients with sepsis and its sequelae. New definitions were offered for some terms, while others were discarded. Broad definitions of sepsis and the systemic inflammatory response syndrome were proposed, along with detailed physiologic variables by which a patient could be categorized. Definitions for severe sepsis, septic shock, hypotension, and multiple organ dysfunction syndrome were also offered. The use of severity scoring methods were recommended when dealing with septic patients as an adjunctive tool to assess mortality. Appropriate methods and applications for the use and testing of new therapies were recommended. The use of these terms and techniques should assist clinicians and researchers who deal with sepsis and its sequelae.
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            Veno-occlusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients.

            To determine the incidence and clinical course of veno-occlusive disease of the liver (VOD) after bone marrow transplantation and to analyze risk factors for severe VOD. Cohort study of 355 consecutive patients. A bone marrow transplantation center. Each patient was prospectively evaluated for VOD, and many risk factors for severe VOD were analyzed using logistic regression models. The relation of VOD to renal and cardiopulmonary failure was analyzed using time-dependent proportional hazards models. Veno-occlusive disease developed in 190 of 355 patients (54%; 95% CI, 48% to 59%): Fifty-four patients had severe VOD and 136 had mild or moderate VOD. Independent variables derived from a multivariate model for predicting severe VOD included elevated transaminase values before transplantation (relative risk, 4.6; P < 0.0001); vancomycin therapy during cytoreductive therapy (relative risk, 2.9; P = 0.003); cytoreductive therapy with a high-dose regimen (relative risk, 2.8; P = 0.01); acyclovir therapy before transplantation (relative risk, 4.8; P = 0.02); mismatched or unrelated donor marrow (relative risk, 2.4; P = 0.02); and previous radiation therapy to the abdomen (relative risk, 2.2; P = 0.04). Vancomycin therapy was a marker for persistent fever. Multiorgan failure was more frequent among patients with VOD and usually followed the onset of liver disease. Veno-occlusive disease, which developed in 54% of bone marrow transplant recipients, is frequently associated with renal and cardiopulmonary failure. Pretransplant transaminase elevations, use of high-dose cytoreductive therapy, and persistent fever during cytoreductive therapy are independent predictors of severe VOD.
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              Venoocclusive disease of the liver following bone marrow transplantation.

              Review of 235 consecutive patients undergoing bone marrow transplantation was performed in order to define the clinical syndrome of venoocclusive disease of the liver (VOD) in these patients. Analysis of all patients with histologically proven VOD revealed a consistent clinical syndrome of liver dysfunction occurring within the first 3 weeks after marrow infusion. This was characterized by hyperbilirubinemia peaking at greater than or equal to 2 mg/dl with at least 2 of 3 other findings: hepatomegaly, ascites, and 5% or greater weight gain. VOD developed in 22% (52 of 235). A persistently elevated aspartate aminotransferase (SGOT) prior to transplant was associated with an increased risk of developing VOD by multivariate analysis (P = 0.0003), and acute leukemia in first remission was associated with a decreased risk (P = 0.02). Neither the preparative regimen (busulfan and cyclophosphamide versus cyclophosphamide and total body irradiation) nor the type of graft (allogeneic versus autologous) influenced the occurrence. Twenty-four of these 52 patients (47%) died with VOD (10% of the entire group). This makes VOD the third leading cause of death in our allogeneic graft recipients, and the second leading cause in our patients receiving autologous transplants. VOD is a common complication of bone marrow transplantation and has a specific clinical presentation, which usually allows diagnosis without the need of liver biopsy.
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                Author and article information

                Journal
                Bone Marrow Transplant
                Bone Marrow Transplant
                Bone Marrow Transplantation
                Nature Publishing Group
                0268-3369
                1476-5365
                July 2016
                16 May 2016
                : 51
                : 7
                : 906-912
                Affiliations
                [1 ]Hematology Department, Hôpital Saint Antoine, and Université Pierre & Marie Curie , Paris, France
                [2 ]Instituto Portugues de Oncologia , Lisbon, Portugal
                [3 ]Department of Haematology, University Hospital Zurich , Zurich, Switzerland
                [4 ]King Abdullah International Medical Research Center/King Saud Bin Abdulaziz University for Health Sciences, Department of Oncology, King Abdulaziz Medical City, Ministry of National Guard Health Affairs , Riyadh, Saudi Arabia
                [5 ]King Faisal Specialist Hospital and Research Centre , Riyadh, Saudi Arabia
                [6 ]Sisli Florence Nightingale Hospital, HSCT Unit , Istanbul, Turkey
                [7 ]Division for Stem Cell Transplantation and Immunology, Department for Children and Adolescents, University Hospital, Goethe University , Frankfurt/Main, Germany
                [8 ]Department of Hematology, University of Liege , Liege, Belgium
                [9 ]Department of Internal Medicine, American University of Beirut , Beirut, Lebanon
                [10 ]Blood and Marrow Transplantation Program, Hematology Department, Institut Paoli-Calmettes , Marseille, France
                [11 ]Hematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute , Milano, Italy
                [12 ]Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Regensburg , Regensburg, Germany
                [13 ]Department of Hematology and Immunology, Hospital Robert Debre, Paris 7-Paris Diderot University , Paris, France
                [14 ]Department of Clinical Haematology, Central Manchester Foundation Trust , Manchester, UK
                [15 ]HSCT division, National Cancer Center Hospital , Tokyo, Japan
                [16 ]Hematology Department, Institut Universitaire du Cancer Toulouse – Oncopole , Toulouse, France
                [17 ]Department of Hematology and Stem Cell Transplantation, St Istvan and St Laszlo Hospital, Budapest 3 Department of Internal Medicine, Semmelweis University , Budapest, Hungary
                [18 ]Hematology Department, Groupement Hospitalier Sud, Hospices Civils de Lyon, Université Claude Bernard Lyon EST , Pierre Bénite, France
                [19 ]Hematology and Bone Marrow Transplantation, Chaim Sheba Medical center , Tel-Hashomer, Israel
                [20 ]National Stem Cell Transplant Unit (Adults), Department of Haematology, St James's Hospital and Academic Department of Haematology, Trinity College Dublin , Dublin, Ireland
                [21 ]Division of Hematology, Department of Medicine, Keio University School of Medicine , Tokyo, Japan
                [22 ]Department of Haematology, King's College Hospital , London, UK
                [23 ]Department of Pediatrics, St Anna Kinderspital , Vienna, Austria
                [24 ]LDS Hospital, Salt Lake City , UT, USA
                [25 ]Division of Hematologic Malignancy, Department of Medical Oncology, Dana-Farber Cancer Institute , Boston, MA, USA
                [26 ]Comprehensive Cancer Center, Helsinki University Hospital , Helsinki, Finland
                [27 ]Hematology and Stem Cell Transplantation Section, Division of Hematology/Oncology, Department of Medicine, Vanderbilt University Medical Center and Veterans Affairs Medical Center , Nashville, Tennessee
                [28 ]Section of Haematology and Coagulation, Department of Internal Medicine, Sahlgrenska University Hospital , Göteborg Sweden
                [29 ]CHU de Lille, LIRIC INSERM U995, université de Lille2 , France
                [30 ]Department of Hematology, Hospital Universitario Puerta de Hierro Majadahonda , Madrid, Spain
                [31 ]Josep Carreras Foundation & Leukemia Research Institute, Hematology Department, Hospital Clínic , Barcelona, Spain
                Author notes
                [* ]Department of Clinical hematology and Cellular therapy, Hôpital Saint Antoine , 184 rue du Faubourg Saint Antoine, Paris 75571 Cedex 12, France. E-mail: mohamad.mohty@ 123456inserm.fr
                [32]

                These authors contributed equally to this work.

                Author information
                http://orcid.org/0000-0003-0369-8515
                Article
                bmt2016130
                10.1038/bmt.2016.130
                4935979
                27183098
                b8d2cc70-bcd6-469f-805d-99ab84b578dc
                Copyright © 2016 Macmillan Publishers Limited

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License. The images or other third party material in this article are included in the article's Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/

                History
                : 17 March 2016
                : 30 March 2016
                : 01 April 2016
                Categories
                Special Report

                Transplantation
                Transplantation

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