How I manage sinusoidal obstruction syndrome after haematopoietic cell transplantation.
Sinusoidal obstruction syndrome (SOS), also called veno-occlusive disease of the liver, is one of the most relevant complications of endothelial origin that appears early after haematopoietic cell transplantation (HCT). Despite its relatively low incidence and the fact that most cases of SOS resolve spontaneously, the cases that evolve to multi-organ failure (MOF; severe SOS) have a mortality rate higher than 80% and represent one of the major clinical problems after HCT. For this reason, transplantation teams must have a pre-established policy regarding preventive measures in high-risk patients, strict daily control of weight and fluid balance during HCT, homogeneous diagnostic criteria, appropriate complementary studies for a correct differential diagnosis and measures to prevent and manage hepatorenal syndrome; in addition they must also be ready to start early treatment with defibrotide in patients with a possible severe SOS. Due to the lack of definitive evidence to enable the establishment of general recommendations in the management of SOS, this review analyses all of these aspects based on the author's personal experience.
Haematology Department, Hospital Clinic, Barcelona, Spain; Spanish Bone Marrow Donor Program, Josep Carreras Foundation, Barcelona, Spain; Josep Carreras Leukaemia Research Institute, Barcelona, Spain.
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Carreras
D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D019468:Disease Management; D004359:Drug Therapy, Combination; D042241:Early Diagnosis; D004730:Endothelium, Vascular / Q000187:drug effects / Q000473:pathology; D005440:Fluid Therapy; D018380:Hematopoietic Stem Cell Transplantation; D006504:Hepatic Veno-Occlusive Disease / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D008775:Methylprednisolone / Q000627:therapeutic use; D009102:Multiple Organ Failure / Q000209:etiology / Q000517:prevention & control; D011089:Polydeoxyribonucleotides / Q000627:therapeutic use; D012307:Risk Factors; D063189:Symptom Assessment; D019172:Transplantation Conditioning / Q000009:adverse effects* / Q000379:methods
D011089:Polydeoxyribonucleotides; C036901:defibrotide; D008775:Methylprednisolone
defibrotide; early complications post-HCT; endothelial damage; sinusoidal obstruction syndrome; veno-occlusive disease of the liver