IngentaConnect Splenectomy for the treatment of thrombotic thrombocytopenic purp...

Authors: Kappers-Klunne, M. C.¹; Wijermans, P.²; Fijnheer, R.³; Croockewit, A. J.⁴; Holt, B.⁵; Wolf, J. T. M.⁶; Löwenberg, B.¹; Brand, A.⁷

Source: British Journal of Haematology, Volume 130, Number 5, September 2005 , pp. 768-776(9)

Publisher: Blackwell Publishing

Abstract:

Summary

Plasma 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.

Keywords: thrombotic thrombocytopenic purpura; refractory; relapsing; splenectomy

Document Type: Research article

DOI: 10.1111/j.1365-2141.2005.05681.x

Affiliations: 1: Department of Haematology, Erasmus Medical Centre, Centre Location, Rotterdam 2: HagaZiekenhuis, Department of Internal Medicine, Den Haag 3: Department of Haematology, University Medical Centre Utrecht, Utrecht 4: Department of Haematology, University Medical Centre St Radboud, Nijmegen 5: Department of Trials and Statistics, Erasmus Medical Centre, Daniel den Hoed Cancer Centre, Rotterdam 6: Department of Haematology, University Medical Centre Groningen, Groningen 7: Department of Haematology and Sanquin Bloodbank South West Region, Leiden University Medical Centre, Leiden, the Netherlands

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Splenectomy for the treatment of thrombotic thrombocytopenic purpura