Veno-occlusive Hepatic Disease (Sinusoidal Obstruction Syndrome) Differential Diagnoses

Updated: Apr 24, 2021
  • Author: James L Harper, MD; Chief Editor: Jennifer Reikes Willert, MD  more...
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DDx

Diagnostic Considerations

Two conflicting definitions of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS), the Seattle criteria and the Baltimore criteria, have been in use since the 1980s. These criteria predict VOD/SOS with an accuracy of more than 90% but have a relatively low sensitivity of 56%. [27]

According to the modified Seattle criteria, 2 or more of the following must be present prior to 20 days after hematopoietic stem cell transplantation (HSCT) for a diagnosis of VOD/SOS:

  • Bilirubin level of more than 2 mg/dL (34 µmol/L)
  • Hepatomegaly and upper right quadrant pain of liver origin
  • Ascites and/or unexplained weight gain of more than 2% above the reference range

According to the Baltimore criteria, hyperbilirubinemia (≥2 mg/dL) and 2 or more of the following must be present prior to 21 days after HSCT:

  • Hepatomegaly (usually painful)
  • Ascites
  • Weight gain of more than 5% above the reference range

More recently, the European Society for Blood and Marrow Transplantation (EBMT) has proposed new diagnostic criteria for VOD/SOS, with separate criteria for pediatric and adult patients. [20, 28] For hepatic VOD/SOS in children, the EBMT includes no limitation for time of onset after HSCT. [28] Diagnosis requires the presence of two or more of the following:

  • Unexplained consumptive and transfusion-refractory thrombocytopenia
  • 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
  • Ascites above baseline value
  • Rising bilirubin from a baseline value on 3 consecutive days or bilirubin increase of ≥2 mg/dL within 72 h

The EBMT advises that hepatomegaly and ascites values are best confirmed by imaging. To determine baseline imaging values, the EBMT recommends performing ultrasonography, computed tomography, or magnetic resonance imaging immediately before HSCT.

For VOD/SOS in adult patients, the EBMT proposed separate criteria for classical and late-onset cases. For classical VOD/SOS in the first 21 days after HSCT, the criteria are bilirubin ≥2 mg/dL and two of the following:

  • Painful hepatomegaly
  • Weight gain > 5%
  • Ascites

For late-onset VOD/SOS in adults developing beyond 21 days after HSCT, the EBMT diagnostic criteria are as follows [20] :

  • Presence of the classical VOD/SOS criteria or
  • Histologically proven SOS/VOD or
  • Two or more of the following: Bilirubin ≥2 mg/dL (34 μmol/L), painful hepatomegaly, weight gain > 5%, ascites and
  • Hemodynamic and/or ultrasonographic evidence of VOD/SOS

Differential Diagnoses