According to a study of the rates of complications and mortality associated with the management of acute immune thrombotic thrombocytopenic purpura (iTTP), most cases of therapeutic plasma exchange (TPE)-related mortality were associated with a delay in the diagnosis of iTTP, highlighting the critical need for rapid identification of patients with iTTP and initiation of TPE. Moreover, although rates of TPE-associated adverse events were high, most complications were treatable.

The retrospective study, which was published in Transfusion, used data from a multi-institutional cohort of adult patients (median age, 42 years; male, 31%; non-Caucasian, 42%) presenting with acute iTTP between 2004 and 2017.

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Of 109 patients included, 8 patients died (7.3%); given 219 captured episodes of acute iTTP in this cohort, the mortality rate per episode was 3.7%. Most deaths occurred within 90 days of a first episode of iTTP (75%, 6/8), though 2 patients died during a TTP relapse. Mortality was not predicted by either the number of TPE treatments or length of hospital stay. For 5 of the 8 patients who died, a delay in the diagnosis of iTTP or initiation of TPE had occurred (1 arrived to the hospital in a moribund state). 

Among 74 patients included in the assessment of complications representing 101 treatment courses, 76% of patients had at least 1 complication associated with TPE. Adverse events were reported in 34% of treatment courses, with the most frequent being mild allergic (urticarial) transfusion reactions that required only diphenhydramine treatment. Severe complications occurred during 6.9% of the treatment courses, including anaphylaxis in 4 cases, thrombosis in 1 case, and line-associated infections in 2 cases (1 death occurred from line-associated bacteremia after 25 TPE procedures).

“Most deaths occurred at first diagnosis among patients who presented with significant end-organ injury or for whom treatment with TPE was delayed,” wrote the researchers. “Refractoriness to treatment with TPE was not a major cause of death. Our data highlight the importance of early identification and rapid up-front use of TPE in patients with suspected iTTP.”

Reference

1.     Colling M, Sun L, Upadhyay V, et al. Deaths and complications associated with the management of acute immune thrombotic thrombocytopenic purpura [published online February 21, 2020]. Transfusion. doi:10.1111/trf.15721