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Multiple diagnostic criteria have been utilized to recognize and diagnose VOD

Traditionally, VOD has been characterized by these signs and symptoms1,2

  • Weight gain
  • Hepatomegaly or right upper quadrant pain
  • Elevated bilirubin
  • Ascites
Early signs suggestive of VOD include increased need for platelet transfusion and fluid retention causing weight gain3

Historically, 2 different criteria have been used for VOD diagnosis1

Modified Seattle criteria

Presentation by Day 20 post HSCT of at least 2 of the following:

  • Bilirubin >2 mg/dL
  • Hepatomegaly or right upper quadrant pain
  • Weight gain (>2%)

Baltimore criteria

Presentation of bilirubin >2 mg/dL by Day 21 post HSCT and at least 2 of the following:

  • Painful hepatomegaly
  • Ascites
  • Weight gain (>5%)

Signs and symptoms of VOD occurring after the first 21 days post HSCT are not considered2,4

VOD that presents in the absence of specified signs and symptoms, such as hyperbilirubinemia, is not considered2,4

EBMT has proposed new criteria for diagnosing VOD in adults and children

Patient icon

Revised EBMT criteria for adults2

VOD that occurs ≤21 days post HSCT
Baltimore criteriaa:

Presentation of bilirubin >2 mg/dL AND at least 2 of the following:

  • Painful hepatomegaly
  • Ascites
  • Weight gain (>5%)

Late onset VOD >21 days post HSCT
Baltimore criteriaa beyond Day 21
OR histologically proven VOD
OR 2 or more of the following criteria must be present:

  • Bilirubin ≥2 mg/dL (or 34 µmol/L)
  • Painful hepatomegaly
  • Weight gain (>5%)
  • Ascites

AND hemodynamic or/and ultrasound evidence of VOD (hepatomegaly, ascites, and decrease in velocity or reversal of portal flow)

VOD that presents in the absence of specified signs and symptoms, such as hyperbilirubinemia, is not considered2,4

Infant icon

Revised EBMT criteria for children5

No limitation for time of onset of VOD

The presence of 2 or more of the following is requiredb:

  • Unexplained consumptive and transfusion-refractory thrombocytopeniac
  • Otherwise unexplained weight gain on 3 consecutive days, despite the use of diuretics, or a weight gain >5% above baseline value
  • Hepatomegaly above baseline value (best if confirmed by imaging)d
  • Ascites above baseline value (best if confirmed by imaging)d
  • Rising bilirubin from a baseline value on 3 consecutive days or bilirubin ≥2 mg/dL within 72 hours

Proposed EBMT criteria have not been prospectively validated in clinical trials

VOD is a clinical diagnosis
  1. Defined as classical VOD in EBMT criteria.
  2. With the exclusion of other potential differential diagnoses.
  3. ≥1 weight-adjusted platelet substitution/day to maintain institutional transfusion guidelines.
  4. Suggested: imaging (US, CT, or MRI) immediately before HCT to determine baseline value for both hepatomegaly and ascites.

CT=computed tomography; EBMT=European Society for Blood and Marrow Transplantation; HCT=hematopoietic cell transplantation; HSCT=hematopoietic stem-cell transplantation; MRI=magnetic resonance imaging; US=ultrasonography; VOD=veno-occlusive disease (also known as sinusoidal obstruction syndrome, or SOS).

References: 1. Carreras E. Early complications after HSCT. In: Apperley J, Carreras E, Gluckman E, et al, eds. The EBMT Handbook. 6th ed. Paris, France: European School of Haematology; 2012:176-195. 2. Mohty M, Malard F, Abecassis M, et al. 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. Bone Marrow Transplant. 2016;51(7):906-912. 3. Bajwa RPS, Mahadeo KM, Taragin BH, et al. Consensus report by Pediatric Acute Lung Injury and Sepsis Investigators and Pediatric Blood and Marrow Transplantation Consortium Joint Working Committees: supportive care guidelines for management of veno-occlusive disease in children and adolescents, part 1: focus on investigations, prophylaxis, and specific treatment. Biol Blood Marrow Transplant. 2017;23(11):1817-1825. 4. Mohty M, Malard F, Abecassis M, et al. Sinusoidal obstruction syndrome/veno-occlusive disease: current situation and perspectives—a position statement from the European Society for Blood and Marrow Transplantation (EBMT). Bone Marrow Transplant. 2015;50(6):781-789. 5. Corbacioglu S, Carreras E, Ansari M, et al. 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. Bone Marrow Transplant. 2018;53(2):138-145.