Defibrotide Dose-escalation for SOS Post-HSCT
Sinusoidal Obstruction Syndrome, Veno-occlusive Disease
About this trial
This is an interventional treatment trial for Sinusoidal Obstruction Syndrome
Eligibility Criteria
Inclusion Criteria: HCT recipients (Auto or Allograft) SOS/VOD as defined by Cairo/Cooke Diagnostic criteria (1) (Table 3) with either renal and/or pulmonary dysfunction as defined by Cairo/Cooke Grading criteria (1) (Appendix I). Unresponsive to standard defibrotide therapy as defined by at least one of the following: Patients with SOS/VOD failing to obtain a complete response (CR) defined by Grade I or less by Cairo/Cooke Grading criteria (1) (Appendix I). This would therefore include patients with stable disease after at least 14 days of defibrotide or partial response after at least 21 days of defibrotide (25mg/kg/day). Progressive disease defined by progression of at least one grade or more from diagnostic grade as defined by Cairo/Cooke Grading criteria (1) (Appendix I) following at least 7 days of defibrotide (25mg/kg/day). Age 1 month - 75 years Exclusion Criteria: Patients who did not receive HCT. Concomitant systemic anticoagulation (excluding central venous line management, fibrinolytic instillation for central venous line occlusion, management of intermittent dialysis or ultrafiltration of CVVH). Active bleeding and/or hemorrhage of at least grade 2 and above. History of development of Grade III/IV anaphylaxis probably or directly secondary to defibrotide. Female patients who are pregnant or breast feeding.
Sites / Locations
Arms of the Study
Arm 1
Experimental
Defibrotide
7.1.1 HCT recipients with SOS/VOD and renal and/or pulmonary dysfunction with either PR after 21 days of standard doses of defibrotide (25mg/kg/day) or SD, after 14 days of standard doses of defibrotide (25mg/kg/day) progressive disease after 7 days on defibrotide (25mg/kg/day) will undergo intra-patient dose escalation every 4 days until a complete response is obtained up until the highest dose level of 100mg/kg/day at which point an endpoint of CR, PR or SD will be sought (see 7.2 for definition of response) (Maximum of 4 dose levels) (7.1.2):