Defibrotide as a promising treatment for thrombotic thrombocytopenic purpura in patients undergoing bone marrow transplantation

Transplantation-associated thrombotic microangiopathy: twenty-two years later

“…55 In a recently reported case series nine responses were seen in a group of 12 patients with moderate to severe TA-TMA after prolonged treatment, suggesting the need for randomized trials of defibrotide in this disorder. 56 Agents that maintain the normal anticoagulant endothelial surface, such as nitric oxide donors and defibrotide, which inhibit platelet aggregation and promote vasodilation, may also alter the course of this disease in bone marrow transplant recipients. 57,58 …”

Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT

“…3,[7][8][9] Treatment of TA-TMA has included withdrawal of offending agents, plasmapheresis, 10 rituximab 11 and defibrotide. 12 However, responses are variable and often unsatisfactory and mortality rates can approach 60%. 3,13 TA-TMA is less commonly reported following auto-SCT.…”

Remission of Microangiopathy in Transplanted Thalassemic Child

“…Considering the effectiveness of Defibrotide (Procyclide) which acts as an anti thrombotic and fibrinolytic drug in post transplant veno-occlusive disease [14,15] pathogenesis related to endothelial damage, we are in favour of this drug for post transplant TAM as claimed by some authors [2,[15][16][17].…”