Hepatic sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life-threatening complication of hematopoietic cell transplantation (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%.
EBMT diagnostic criteria for hepatic SOS/VOD in children
- No limitation for time of onset of SOS/VOD
The presence of two or more of the followinga
- Unexplained consumptive and transfusion-refractory thrombocytopeniab
- Otherwise unexplained weight gain on three consecutive days despite the use of diuretics or a weight gain >5% above baseline value
- cHepatomegaly (best if confirmed by imaging) above baseline value
- cAscites (best if confirmed by imaging) above baseline value
- Rising bilirubin from a baseline value on 3 consecutive days or bilirubin ≥2 mg/dL within 72 h
Abbreviations: CT= computed tomography; HCT= hematopoietic cell transplantation; MRI = magnetic resonance imaging; SOS/VOD= sinusoidal obstruction
syndrome/veno-occlusive disease; US=ultrasonography; EBMT=European Society for Blood and Marrow Transplantation
a With the exclusion of other potential differential diagnoses.
b ≥ 1 weight-adjusted platelet substitution/day to maintain institutional transfusion guidelines.
c Suggested: imaging (US, CT or MRI) immediately before HCT to determine baseline value for both hepatomegaly and ascites.
EBMT criteria for grading the severity of suspected hepatic SOS/VOD in children
|CTCAE||Mild||Moderate||Severe||Very severe MOD/MOF|
|LFTb (ALT, AST, GLDH)||≤2 × normal||>2 and ≤5 × normal||>5||>5|
|Persistent RTb||<3 days||3–7 days||>7 days||>7 days|
|Ascitesb||Minimal||Moderate||Necessity for paracentesis (external drainage)|
|Bilirubin kinetics||Doubling within 48 h|
|Coagulation||Normal||Normal||Impaired coagulation||Impaired coagulation with need for replacement of coagulation factors|
|Renal function GFR (mL/min)||89–60||59–30||29–15||<15 (renal failure)|
|Pulmonary function (oxygen requirement)||<2 L/min||>2 L/min||Invasive pulmonary ventilation (including CPAP)|
|CNS||Normal||Normal||Normal||New onset cognitive impairment|
Abbreviations: ALT= alanine transaminase; AST= aspartate transaminase; CNS =central nervous system; CPAP=continuous positive airway pressure;
CTCAE =Common Terminology Criteria for Adverse Events; GFR =glomerular filtration rate; GLDH = glutamate dehydrogenase; LFT =liver function test; MOD/
MOF= multi-organ dysfunction/multi-organ failure; RT=refractory thrombocytopenia; SOS/VOD, sinusoidal obstruction syndrome/veno-occlusive disease.
If patient fulfills criteria in different categories they must be classified in the most severe category. In addition, the kinetics of the evolution of cumulative
symptoms within 48 h predicts severe disease.
b Presence of ≥ 2 of these criteria qualifies for an upgrade to CTCAE level 4 (very severe SOS/VOD).
c Excluding pre-existent hyperbilirubinemia due to primary disease.
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Created Jun 12, 2019.