Abstract Number: PB1868
Meeting: ISTH 2020 Congress
Background: Thrombotic thrombocytopenia purpura (TTP) still presents a significant clinical challenge for physicians and despite increased awareness TTP remains a serious life-threatening illness with current mortality rates estimated at 10%-20%. The recently introduced PLASMIC score enables rapid identification of TTP based on readily available clinical and was suggested to correlate with patient outcome. However, additional validation of this practical clinical prediction tool is warranted to determine its applicability in prediction of patient outcome.
Aims: Determine whether the PLASMIC score correlates with outcome of TTP patients as a whole and specifically in patients who experienced TTP following a prior allogeneic stem cell transplantation (allo-SCT).
Methods: This was a retrospective analysis consisting of all consecutive adult TTP patients treated in the Chaim Sheba Medical Center in 2008-2018. Baseline clinical and laboratory data were analyzed for the cohort as a whole and then specifically for patients who underwent a prior allogeneic stem cell transplantation (allo-SCT).
Results: In all, our cohort consisted of 89 patients with a median age of 53 years (range 18-73 years), 52 patients (58%) underwent a prior allo-SCT. Plasma exchange therapy was initiated in 32 patients (36%), rituximab was given to 16 patients (18%), and aspirin was administered to 12 patients (14%). Transplanted patients were significantly more likely to experience relapse and TTP related mortality compared to non-transplanted patients, 74% vs. 46% (p=0.013), and 81% vs. 28% (p< 0.0001), respectively. Transplanted patients had a significantly lower mean PLASMIC score compared to non-transplanted patients, 4.76 versus 5.5 (p< 0.0001) whereas patients with lower PLASMIC scores experienced increased TTP related mortality only in the post-transplant setting (p=0.001).
Conclusions: Our findings reaffirm the prognostic role of PLASMIC score in predicting outcome of TTP patients including in the setting of post-transplant TTP.
To cite this abstract in AMA style:Cohen Y, Miller L, Mandel M, Keren N, Keren-Politansky A, Avigdor A, Shimoni A, Nagler A, Yonath H, Canaani J. PLASMIC Score Predicts Outcome of Transplanted Patients with Severe Thrombotic Thrombocytopenic Purpura [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/plasmic-score-predicts-outcome-of-transplanted-patients-with-severe-thrombotic-thrombocytopenic-purpura/. Accessed October 2, 2023.
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