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Bone Marrow Transplant. 2018 Feb;53(2):138-145. doi: 10.1038/bmt.2017.161. Epub 2017 Jul 31.

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.

Author information

1
Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Regensburg, Regensburg, Germany.
2
Hematology Department, Josep Carreras Foundation & Leukemia Research Institute, Hospital Clínic, Barcelona, Spain.
3
Hemato-Oncology Unit, Department of Pediatrics, University Hospital of Geneva, Geneva, Switzerland.
4
Pediatric Clinic, University of Milano-Bicocca, San Gerardo Hospital, Milan, Italy.
5
Department of Pediatric Oncohematology, Giambattista Rossi University Hospital, Verona, Italy.
6
Department of Hematology and Immunology, Hospital Robert Debre, Paris 7-Paris Diderot University, Paris, France.
7
Department of Clinical Haematology, Manchester Royal Infirmary, Manchester, UK.
8
Royal Hospital for Sick Children, Glasgow, UK.
9
Division of Blood and Marrow Transplantation, University Children's Hospital, Zurich, Switzerland.
10
Department of Pediatrics, University Hospital of Jena, Jena, Germany.
11
Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.
12
Department of Pediatric Hematology and Oncology, University of Pavia, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
13
Department of Haematology, King's College Hospital, London, UK.
14
Department of Pediatrics, St Anna Kinderspital, Vienna, Austria.
15
Division of Hematologic Malignancy, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
16
Department of Pediatrics, University Children's Hospital, Ulm, Germany.
17
Department of Pediatrics, University Hospital Motol, Prague, Czech Republic.
18
Schneider Children's Medical Center of Israel and Sackler Faculty of Medicine, University of Tel Aviv, Tel Aviv, Israel.
19
Pediatric Hematology-Oncology, Children's Hospital, Medical School, Hannover, Germany.
20
Skåne University Hospital, Lund, Sweden.
21
University Hospital and Polytechnic La Fe, Valencia, Spain.
22
Children's Hospital, University of Helsinki, Helsinki, Finland.
23
Department of Pediatric Hematology, Oncology and Hematopoietic Stem Cell Transplantation, University of Medical Sciences, Poznan, Poland.
24
Section of Hematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden.
25
Royal Manchester Children's Hospital, Manchester, UK.
26
Pediatric Stem Cell Transplantation Unit, Bahçeşehir University School of Medicine, Istanbul, Turkey.
27
Hôpital Saint-Antoine, APHP, Université Pierre & Marie Curie, INSERM UMRS 938, Paris, France.
28
Division for Stem Cell Transplantation and Immunology, Department for Children and Adolescents, University Hospital, Goethe University, Frankfurt/Main, Germany.

Abstract

The advances in hematopoietic cell transplantation (HCT) over the last decade have led to a transplant-related mortality below 15%. Hepatic sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life-threatening complication of HCT that belongs to a group of diseases increasingly identified as transplant-related, systemic endothelial diseases. In most cases, SOS/VOD resolves within weeks; however, severe SOS/VOD results in multi-organ dysfunction/failure with a mortality rate >80%. A timely diagnosis of SOS/VOD is of critical importance, given the availability of therapeutic options with favorable tolerability. Current diagnostic criteria are used for adults and children. However, over the last decade it has become clear that SOS/VOD is significantly different between the age groups in terms of incidence, genetic predisposition, clinical presentation, prevention, treatment and outcome. Improved understanding of SOS/VOD and the availability of effective treatment questions the use of the Baltimore and Seattle criteria for diagnosing SOS/VOD in children. The aim of this position paper is to propose new diagnostic and severity criteria for SOS/VOD in children on behalf of the European Society for Blood and Marrow Transplantation.

PMID:
28759025
PMCID:
PMC5803572
DOI:
10.1038/bmt.2017.161
[Indexed for MEDLINE]
Free PMC Article

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