Management and outcomes for patients with TTP: analysis of 100 cases at a single institution

Abstract

The advent of plasma exchange has led to a dramatic improvement in the survival of patients with thrombotic thrombocytopenic purpura (TTP), though approximately 10% of patients still die and a third suffer relapses. Clinical features that identify poor risk patients have not been clearly identified. We reviewed 100 patients who were treated for a first episode of TTP at the Cleveland Clinic between 2000 and 2012 to identify factors predictive of poor outcomes. On multivariate analysis, increasing age, especially age > 60 (RR: 7.08, 95% CI: 2.15-23.39, P = 0.002), severe neurological symptoms at presentation (RR: 18.37, 95% CI: I4.19-80.13, P < 0.001) and a persistently elevated LDH level after two plasma exchanges were predictive of mortality. Patients with ADAMTS13 activity above or below 5% did not differ in terms of clinical presentation or mortality and relapse rates, although ADAMTS13 activity > 5% was an independent predictor of adverse renal outcomes (need for dialysis and progression to chronic kidney disease). These variables may be useful for risk stratification and identification of patients who could potentially benefit from early institution of adjunctive therapy.

Clinical presentation in thrombotic thrombocytopenic purpura: Real‐world data from two Mexican institutions

“…The latter was also found in our study; 70% of affected patients were female, and the median of age at diagnosis (across genders) was 38.5 years. However, it is not uncommon that older people present with TTP for the first time and have a higher risk for mortality . Our data showed that 10% of the patients included in this study were at least 60 years old, and all of them died due to TTP progression.…”

Elevated plasma neutrophil elastase concentration is associated with disease activity in patients with thrombotic thrombocytopenic purpura

“…Furthermore, several patients may go into sustained clinical remission despite deficient ADAMTS13 activity [7,8]. In addition, although patients with acute TTP often present without acute disease in history, in the majority of TTP patients infections or pregnancy precede the acute disease flare [9].…”

Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura

“…The platelet count is the generally accepted indicator of disease activity in acquired TTP and guides treatment decisions such as when to stop daily plasma exchange. 11 Symptoms of acquired TTP vary according to the organs affected by the tissue ischemia, 17 reflecting cardiac, 4,5,[18][19][20][21] neurologic, 22 or renal 18,23 injury. The pathogenesis of acquired TTP is mediated by the production of autoantibodies against ADAMTS13, the key factor controlling von Willebrand factor-mediated platelet aggregation, and these autoantibodies are the focus of current treatment.…”