Splenectomy for the treatment of thrombotic thrombocytopenic purpura

British Journal of Haematology - Tập 130 Số 5 - Trang 768-776 - 2005
M. C. Kappers‐Klunne1, P. W. Wijermans2, Rob Fijnheer3, Alexandra Croockewit4, Bronno van der Holt5, J. Th. M. de Wolf6, Bob Löwenberg1, Anneke Brand7
1Department of Haematology, Erasmus Medical Centre, Centre Location, Rotterdam
2HagaZiekenhuis, Department of Internal Medicine, Den Haag
3Department of Haematology University Medical Centre Utrecht, Utrecht
4Department of Haematology, University Medical Centre St Radboud, Nijmegen
5Department of Trials and Statistics, Erasmus Medical Centre, Daniel den Hoed Cancer Centre, Rotterdam
6Department of Haematology, University Medical Centre Groningen, Groningen
7Department of Haematology and Sanquin Bloodbank South West Region, Leiden University Medical Centre, Leiden, the Netherlands

Tóm tắt

SummaryPlasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these conditions but its value remains controversial. We report on a series of 33 patients with TTP who were splenectomised because they were plasma refractory (n = 9) or for relapsed disease (n = 24). Splenectomy generated prompt and unmaintained remissions in all except five patients, in whom remission was delayed (n = 4) or who died with progressive disease (n = 1). Four postoperative complications occurred: one pulmonary embolism and three surgical complications. Median follow‐up after splenectomy was 109 months (range 28–230 months). The overall postsplenectomy relapse rate was 0·09 relapses/patient‐year and the 10‐year relapse‐free survival (RFS) was 70% (95% CI 50–83%). In the patients with relapsing TTP, relapse rate fell from 0·74 relapses/patient‐year before splenectomy to 0·10 after splenectomy (P < 0·00001). Two patients died from first postsplenectomy relapse. Although these results are based on retrospective data and that the relapse rate may spontaneously decrease with time, we conclude that splenectomy, when performed during stable disease, has an acceptable safety profile and should be considered in cases of plasma refractoriness or relapsing TTP to reach durable remissions and to reduce or prevent future relapses.

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Thông tin xuất bản

British Journal of Haematology

Tập 130 Số 5

768-776

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