Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Clinical Features and Risk Factors of Relapse and Mortality in Thrombotic Thrombocytopenic Purpura Patients: A Seven-Year Experience



Shiraji ST1 ; Foumani HK1 ; Mousavi SA1 ; Vaezi M1 ; Rad S1 ; Babakhani D1 ; Barkhordar M1 ; Bahri T1 ; Janbabaei G1 ; Malekmohammadi A1 ; Mohammadi S1 ; Rostami MR1 ; Mansouri V2
Authors
Show Affiliations
Authors Affiliations
  1. 1. Research Institute for Oncology, Hematology and Cell Therapy, Hematology-Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Digestive Diseases Research Institute, Tehran University of Medical Sciences, Tehran, Iran

Source: International Journal of Hematology-Oncology and Stem Cell Research Published:2023

Abstract

Background: Thrombotic thrombocytopenic purpura (TTP) is associated with microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis. No comprehensive report exists on clinical characteristics and risk factors of relapse and mortality in Iranian TTP patients. In this study, we aimed to report clinical features of Iranian TTP patients, to evaluate disease relapse and mortality rate and their associated risk factors. Materials and Methods: This study was a cohort study of patients diagnosed with microangiopathic hemolytic anemia admitted to the Shariati Hospital, Tehran, a referral center for TTP patients, from 2010 to 2017. Demographic, clinical, and laboratory data were recorded and patients were followed for 3 years regarding disease relapse and mortality. Results: 114 patients (80 female, 34 male) with a mean age of 39.3 ± 14.99 years were included. Hematologic and neurologic symptoms were the most common manifestations. Abnormal laboratory findings at the presentation included thrombocytopenia, anemia, and elevated LDH. All patients were treated with plasma exchange, and 75.5% of them had a response to treatment, while the 3-year relapse and mortality rate was 23.6 and 26.3%. Lower platelet count was a predictor of disease relapse. Age, hematological, or neurological initial presentation were associated with TTP mortality. Conclusion: Based on the largest study of TTP patients ever in Iran, the demographic and clinical characteristics of Iranian TTP patients are similar to other existing reports. Knowledge of the risk factors for TTP relapse and mortality could be useful to alert hematologists for prompt therapeutic actions when necessary. © 2023 Tehran University of Medical Sciences.