Real-World Analysis of Clinical Characteristics, Treatment Outcomes, and the Novel Predictive Model for Patients with Thrombotic Thrombocytopenic Purpura (TTP) and TTP-Like Syndrome.

Abstract

Purpose: It is crucial to differentiate critically ill patients exhibiting thrombocytopenia and hemolytic anemia alongside organ damage to enable rapid identification of thrombotic thrombocytopenic purpura (TTP) and TTP-like syndrome, which allows for targeted emergency interventions such as plasma exchange.

Patients and methods: This study retrospectively analyzed clinical data from patients with TTP and TTP-like syndrome to further elucidate the potential differences between these conditions. We also established a new predictive model to facilitate early identification and differentiation between TTP and TTP-like syndrome. A new predictive model for diagnosing TTP was developed using five key indicators: reticulocyte percentage, platelet count, schistocyte percentage, LDH/ULN, and indirect bilirubin. The performance of this new model was compared with the traditional PLASMIC score by evaluating sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV).

Results: Thirty-five patients were diagnosed with TTP and 42 were diagnosed with TTP-like syndrome. TTP is most commonly associated with autoimmune diseases (n=13, 37.14%), while TTP-like syndrome frequently arises from infections (n=23, 54.76%). The ADAMTS13 activity was significantly lower in the TTP group than in the TTP-like syndrome group (Mean 8.30% vs 46.12%). TTP-like syndrome patients had significantly higher levels of inflammatory markers. The new predictive model was developed for TTP with a predictive ability of 96.9%. Overall, 16 patients (20.77%) died, including 3 (8.57%) in the TTP group and 13 (30.95%) in the TTP-like syndrome group. Kaplan-Meier survival analysis showed significant differences in survival between TTP and TTP-like syndrome patients, with a 180-day overall survival (OS) rate of 90.6% vs 60.9% (p=0.009); and plasma exchange improved 180-day OS rate in the TTP group compared to the TTP-like syndrome group (90.6% vs 65.6%) (p=0.054).

Conclusion: This study demonstrates that TTP and TTP-like syndrome are two distinct types of diseases. The new predictive model has shown good efficacy in distinguishing TTP and TTP-like syndrome. Plasma exchange significantly improves survival in TTP patients; however, its effect on TTP-like syndrome is minimal.