Abstract
Objective
This study aims to investigate the clinical characteristics, timing of intervention, diagnostic and treatment options, and prognostic factors in patients with immune thrombotic thrombocytopenic purpura (iTTP).Methods: We conducted a retrospective analysis of iTTP patients who visited the Second Hospital of Jilin University from January 2020 to April 2023, encompassing individuals with complete clinical data. The analysis included an examination of clinical manifestations and auxiliary test results.Results: The study involved 6 patients (5 males, 1 female) with an age range of 29–84 years, and a median age of 58 years. Common clinical manifestations comprised thrombocytopenia, hematuria, soy sauce-colored urine, as well as neurological and psychiatric symptoms. All six patients underwent treatment with therapeutic plasma exchange(TPE), with three of them also receiving rituximab (3/6). The follow-up period ranged from 1 to 21 months, with a median of 10 months, concluding in April 2023. Of the six cases, three patients survived, while the remaining three succumbed in March 2021, July 2022, and March 2023, respectively.Conclusion: TTP diagnosis is often delayed due to its nonspecific clinical presentation. Accurate clinical diagnosis of TTP relies on assessing ADAMTS13 activity levels and genetic testing. Following PLASMIC-S guidelines, immediate plasma exchange and glucocorticoid therapy are recommended once diagnosis is suspected. Additionally, the evolving clinical landscape has seen an increasing number of patients benefiting from a combination of anti-CD20 monoclonal antibodies and other novel drugs. This expanding repertoire of treatment options enhances the multidisciplinary and comprehensive diagnostic and therapeutic strategy for iTTP.