Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS‐13

British Journal of Haematology - Tập 136 Số 3 - Trang 451-461 - 2007
Marie Scully1, Hannah Cohen2, Jamie Cavenagh3, Sylvia Benjamin4, Richard Starke5, Sally Killick6, Ian Mackie5, Samuel J. Machin5
1Department of Haematology, University College London, London, UK
2University College London Hospitals NHS Foundation Trust, London, UK
3St Bartholomew's and the Royal London Hospital, London, UK
4NBS, John Radcliffe Hospital, Oxford, UK
5Departments of Haematology, University College London, London, UK
6Royal Bournemouth and Christchurch Hospitals NHS Foundation Trust, Bournemouth, UK

Tóm tắt

SummaryThrombotic thrombocytopenic purpura (TTP) is a life‐threatening disorder and plasma exchange (PEX) remains the primary treatment modality. Twenty‐five patients with acute refractory/relapsing idiopathic TTP received rituximab in conjunction with PEX because of progressive clinical disease or deterioration in laboratory parameters, despite intensive standard therapy. In relapsing TTP, rituximab was started if antibody to ADAMTS‐13 (a disintegrin and metalloproteinase with thrombospondin motif‐13) was demonstrated during previous episodes. All 25 patients attained complete clinical and laboratory remission in a median of 11 d after initiating rituximab. In 21 cases, ADAMTS‐13 activity was within the normal range following rituximab. Inhibitors were detected in 24/25 patients by mixing studies and/or immunoglobulin G (IgG) antibodies to ADAMTS‐13 pre‐rituximab. There was no evidence of inhibitors and/or IgG activity <10% in 23/25 patients following rituximab. In acute refractory cases, the median number of PEX pre‐rituximab and following the first rituximab infusion was 13 and 9, respectively. There have been no infectious complications, despite low CD 19 levels and no relapses. In patients with acute refractory/relapsing idiopathic TTP, rituximab appears to be a safe, effective, targeted therapy with a significant reduction in the requirement for PEX.

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Thông tin xuất bản

British Journal of Haematology

Tập 136 Số 3

451-461

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