Long-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy.
Author
Doyle, Andrew JStubbs, Matthew J
Dutt, Tina
Lester, Will
Thomas, Will
van Veen, Joost
Hermans, Joannes
Cranfield, Tanya
Hill, Quentin A
Clark, Amanda
Bagot, Catherine
Austin, Steven
Westwood, John-Paul
Thomas, Mari
Scully, Marie
Publication date
2023-01-19Subject
Haematology
Metadata
Show full item recordAbstract
Disease relapse is recognized as a risk in immune-mediated thrombotic thrombocytopenic purpura (iTTP) after treatment of the acute presenting episode. Identification of patients at risk of relapse and its patterns are yet to be clearly established. We reviewed patients with iTTP having had >3 years of follow-up over 10 years in the United Kingdom to identify patient characteristics for relapse, assess relapse rates and patterns, and response to anti-CD20 therapy in those with a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) relapses (ADAMTS13 activity of <20% without thrombocytopenia). We identified 443 patients demonstrating relapse rates of 40% at 5-year follow-up. At 10-year follow-up, no difference in relapse was observed irrespective of whether rituximab was used at acute presentation (P = .39). Black Caribbean ethnicity increased the risk of disease relapse in the British population. There was a distinct population of patients (6%) that relapsed early with subsequent frequent relapses occurring on average within 2 years (average time to relapse in subgroup, 1.7 years). Overall, nearly 60% of relapses described were ADAMTS13 relapses, with subsequent treatment reducing the risk of progression to clinical relapses. We demonstrate that iTTP diagnosed in the latter part of the study period had lower rates of clinical relapses (22.6% vs 11.1%, P = .0004) with the advent of regular monitoring and preemptive rituximab. In ADAMTS13 relapses, 96% responded to anti-CD20 therapy, achieving ADAMTS13 activity of >20%. Anti-CD20 therapy was demonstrated to be an effective long-term treatment regardless of relapse pattern and there was no loss of this treatment response after subsequent treatment episodes.Citation
Doyle AJ, Stubbs MJ, Dutt T, Lester W, Thomas W, van Veen J, Hermans J, Cranfield T, Hill QA, Clark A, Bagot C, Austin S, Westwood JP, Thomas M, Scully M. Long-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy. Blood. 2023 Jan 19;141(3):285-294. doi: 10.1182/blood.2022017023Type
ArticlePMID
36322971Journal
BloodPublisher
Elsevierae974a485f413a2113503eed53cd6c53
10.1182/blood.2022017023