EASIX, a new tool to predict response and refractoriness in immune-mediated thrombotic thrombocytopenic purpura.

ABSTRACT:

Introduction

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy resulting from severe ADAMTS13 deficiency. Caplacizumab accelerates platelet recovery, but ~15% of patients remain refractory, and endothelial/microvascular injury or low ADAMTS13 activity may persist despite remission, highlighting the need for biomarkers. We evaluated the Endothelial Activation and Stress Index (EASIX), an endothelial dysfunction surrogate, dynamics and ability to predict refractoriness and mortality in iTTP.

Methods

Fifty-five adults receiving ≥2 therapies (corticosteroids, plasma exchange, rituximab, and/or caplacizumab) were studied. Clinical and laboratory data were collected at baseline, days 1-2, 7, 14, 21, 28, 35, and at treatment

SUBMITTER: Escribano-Serrat S 

PROVIDER: S-EPMC12665919 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature