Thrombotic thrombocytopenic purpura is associated with a high relapse rate after plasma exchange: a single-centre experience
AuthorFrawley, Natasha; NG, ASHLEY; NICHOLLS, KATHLEEN; Hogan, Chris; COHNEY, SOLOMON; GRIGG, ANDREW
Source TitleInternal Medicine Journal
University of Melbourne Author/sNg, Ashley; Nicholls, Kathleen; Hogan, Christopher; Cohney, Solomon; Grigg, Andrew
AffiliationSchool of Medicine
Document TypeJournal (On-line/Unpaginated)
CitationsFrawley, N., Ng, A., Nicholls, K., Hogan, C., Cohney, S., & Grigg, A. (2008). Thrombotic thrombocytopenic purpura is associated with a high relapse rate after plasma exchange: a single-centre experience. Internal Medicine Journal.
Access StatusOpen Access
This is a pre-print version of an article submitted for publication in Internal Medicine Journal. doi:10.1111/j.14455994.2008.01637.x http://www.blackwellpublishing.com/journal.asp?ref=1444-0903&site=1
Background: Thrombotic thrombocytopenic purpura (TTP) is a rare condition characterized by microangiopathic haemolytic anaemia, thrombocytopenia, renal and/or neurological dysfunction secondary to microvascular or macrovascular thrombosis. Despite advances in treatment, TTP remains a serious condition with signiﬁcant morbidity and mortality. Methods: We undertook an audit of patients with TTP over 14 years to assess remission, relapse, survival and factors predictive of outcome using current therapy based on plasma exchange with fresh-frozen plasma. Results: Forty patients were identiﬁed between January 1992 and December 2005. Thirty-one (82%) achieved complete response (CR) to therapy using plasma exchange with fresh-frozen plasma (median 11 exchanges) and steroids. Twelve (37%) relapsed a median of 14 days following cessation of therapy, with multiple relapses occurring in two patients. TTP-related death occurred in four patients during their initial presentation and in two during subsequent relapse. Four patients were only partially responsive to ﬁrst-line therapy. The absence of neurological features at presentation was the only factor predicting a sustained CR to ﬁrst-line therapy (P = 0.027, log–rank analysis). The mean duration of inpatient treatment was 18 days (range 4–38 days) with 30% of patients requiring intensive care admission. Thirty-four per cent of patients acquired central venous line infection, with a median of two episodes of line sepsis per patient. Conclusion: Our results indicate the need for better treatments to reduce the high early relapse rate and signiﬁcant mortality associated with current therapy.
Keywordsthrombotic thrombocytopenic purpura; neurological feature; plasma exchange; steroid; rituximab.
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