Posttransplantation Thrombotic Thrombocytopenic Purpura: A Single-Center Experience and a Contemporary Review

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

“…Our findings are in concordance with previous publications of use of PE in TA-TMA, in which significant response rates to PE have been reported. [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Of note, all patients in our series had multifactorial fulminant TA-TMA, a subgroup of TA-TMA initially reported to be unresponsive to PE. 4,10 However, subsequent experience suggested that mild Grades (0-2) of multifactorial fulminant TA-TMA may indeed respond to PE.…”

“…2,3,8 Unlike idiopathic TTP, TA-TMA has only rarely been associated with severe deficiency of the plasma protease ADAMTS13. 8,[27][28][29] Current therapeutic recommendations of TA-TMA revolve around treatment of and/or elimination of conditions resulting in endothelial insult. 2,3 These recommendations include immediate cessation of potential culprit medications and aggressive treatment of associated GVHD and/or infection.…”

“…2,3,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] This overall poor response to PE in TA-TMA is not necessarily surprising in light of recent insights into its pathogenesis, which suggest that TA-TMA is, in fact, a multifactorial disorder resulting from endothelial cell dysfunction secondary to a variety of insults, including immunosuppressive medications, GVHD and (especially viral) infection. 2,3,8 Unlike idiopathic TTP, TA-TMA has only rarely been associated with severe deficiency of the plasma protease ADAMTS13. 8,[27][28][29] Current therapeutic recommendations of TA-TMA revolve around treatment of and/or elimination of conditions resulting in endothelial insult.…”

“…2,3,24 However, this belies the fact that significant clinical responses have been reported in a significant minority of patients treated with PE in a majority of published TA-TMA series to date. [1][2][3][4][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] Owing to this historic experience, in many transplant centres, including our own, PE continues to be used in the management of TA-TMA. In an attempt to determine which clinical factors may be predictive of response (or lack thereof) to PE, we retrospectively reviewed the outcomes of TA-TMA treated with therapeutic PE at our institution.…”

“…[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.…”