Clinical Features and Risk Factors of Relapse and Mortality in Thrombotic Thrombocytopenic Purpura Patients: A Seven-Year Experience

Autor: Sahar Tavakoli Shiraji, Hosein Kamranzadeh Foumani, Seied Asadollah Mousavi, Mohammad Vaezi, Soroush Rad, Davood Babakhani, Maryam Barkhordar, Tanaz Bahri, Ghasem Janbabaei, Ashraf Malekmohammadi, Saeed Mohammadi, Mohammad Reza Rostami, Vahid Mansouri
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: International Journal of Hematology-Oncology and Stem Cell Research, Vol 17, Iss 3 (2023)
Druh dokumentu: article
ISSN: 2008-2207
84714964
DOI: 10.18502/ijhoscr.v17i3.13305
Popis: 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.
Databáze: Directory of Open Access Journals
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