Risk Factors and Severe Outcome in Thrombotic Microangiopathy After Allogeneic Hematopoietic Stem Cell Transplantation

Abstract

The study underlines the possibility of finding out which patients are more prone to developing post-HSCT TMA, and identifies which risk factors are more frequently associated with a dismal outcome after TMA.

Transplantation-associated thrombotic microangiopathy: effect of concomitant GVHD on efficacy of therapeutic plasma exchange

“…[1][2][3][4][5][6][7] This uncertainty in diagnosis has led to wide variations in the reported incidence of TA-TMA, a lack of consensus regarding clinical approach to management, and poor understanding of prognostic factors with respect to therapeutic response and survival. [1][2][3][4][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] To address this issue of diagnostic clinical uncertainty, two new diagnostic criteria for TA-TMA have recently been proposed; the Bone Marrow Transplant Clinical Trials Network (BMT-CTN) and the IWG (International Working Group) TA-TMA criteria. 24,25 However, neither criterion has been rigorously evaluated clinically, and issues remain with respect to the diagnostic sensitivity of both.…”

Remission of Microangiopathy in Transplanted Thalassemic Child

“…Some authors are in favour of complete stopping of medications such as CSA, Tacrolimus and Sirolimus as soon as TAM is diagnosed [3,4] but no reliable data have supported this suggestion [2]. Notably in our patient resumption of full dose CSA did not result in TAM relapse.…”

“…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].…”

“…Post-transplant TAM is probably due to the presence of a great amount of microaggregates of ultra large von willebrand factors (ULvWFs) in the microcirculation and consequent hemolytic anemia and organ damage. Many factors can contribute to TAM onset, such as severe GVHD, infection (fungal, viral) or the use of cyclosporine a (CSA) or Tacrolimus, all conditions which can result in severe endothelial damage and extensive generation of ULvWFs in the microcirculation [1,2].…”

“…The TAM usually occurs within the first 100 days after stem cell transplantation and is often associated with adult recipient age, female sex, unrelated or HLA-mismatched donor grafts, GVHD [2]. The incidence of TAM is variable, depending on the diagnostic difficulty, and varies 6-12 % after allogeneic transplantation and 1-5 % after autologous transplantation [3][4][5][6][7][8] with mortality rates as high as 30-50 %.…”