DISEASE CHARACTERISTICS:

• Clinical diagnosis of hepatic veno-occlusive disease (VOD), as indicated by jaundice (i.e., bilirubin > 2 mg/dL) AND meets ≥ 2 of the following criteria by day 21 post hematopoietic stem cell transplantation (HSCT):

  • Ascites
  • Weight gain ≥ 5% above baseline weight (i.e., weight on the first day of HSCT conditioning or admission to the HSCT unit)

  • Hepatomegaly

    • Patients with preexisting hepatomegaly must have increased liver size compared to baseline (i.e., time of admission for HSCT), documented by physical exam or imaging

• Severe disease with multiorgan failure, as evidenced by any of the following by day 28 post HSCT:

  • Renal dysfunction, defined by any of the following:

    • Creatinine ≥ 3 times the value on the date of admission for HSCT conditioning OR ≥ 3 times the lowest value during conditioning prior to HSCT (whichever is lower)
    • Creatinine clearance or glomerular filtration rate ≤ 40% of admission value
    • Dialysis-dependent

  • Pulmonary dysfunction attributable to fluid overload or mechanical impingement from abdominal distention or hepatic enlargement not due to an infectious cause (e.g., documented pneumonia), as evidenced by any of the following:

    • Documented oxygen saturation ≤ 90% on room air
    • Requires oxygen supplementation
    • Ventilator-dependent
• No alternative diagnosis (e.g., fulminant viral hepatitis) for ascites, weight gain, and jaundice at the time that severe VOD diagnosis criteria are met

PATIENT CHARACTERISTICS:

• No documented, preexisting (at the time of HSCT) cirrhosis of the liver

• No documented diagnosis of grade B-D graft-versus-host disease (GVHD) involving the liver or gut or documented grade C or D GVHD involving skin of > 50% of body surface

  • Grade B GVHD involving skin only is allowed
• No clinically significant uncontrolled acute hemorrhage requiring >15 cc/kg of PRBC transfusion
• No bleeding from a potentially life-threatening source (e.g., pulmonary hemorrhage or CNS bleeding), irrespective of the amount of blood loss, at any point from the date of HSCT through the date of severe VOD diagnosis

• No hemodynamic instability requiring multiple pressors

  • Able to maintain mean arterial pressure (MAP) with single-pressor support

    • Patients on a single pressor must have stable MAP ≥ 8 hours
    • Patients requiring renal dose dopamine alone (2-5 mcg/kg/min) are eligible without measuring the MAP
    • Pediatric patients must be able to maintain MAP within 1 standard deviation of age-adjusted levels

• Not oxygen-dependent during conditioning prior to HSCT

  • Transient dependence that resolves allowed
• Not dialysis-dependent from admission to completion of HSCT

PRIOR CONCURRENT THERAPY:

• See Disease Characteristics
• No prior solid organ transplantation
• At least 12 hours since prior and no concurrent heparin or other anticoagulants unless used for routine central venous line management, fibrinolytic instillation for central venous line occlusion, intermittent dialysis, or ultrafiltration of continuous veno-venous hemofiltration
• No other concurrent medication that would increase the risk of hemorrhage (e.g., warfarin, nonsteroidal anti-inflammatory drugs, heparin, or systemic tissue-type plasminogen activator)
• No concurrent antithrombin III
• Packed red blood cell (PRBC) transfusion in the absence of clinically significant uncontrolled acute bleeding (e.g., for dialysis or hemodilution) allowed

• Concurrent ursodiol allowed for prophylaxis if initiated at time of HSCT

  • Concurrent initiation of ursodiol allowed for gall bladder sludging
• No re-enrollment onto this trial for patients needing retreatment with defibrotide for recurrent VOD