Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpura. Aqui NA, Stein SH, Konkle B, Abrams CS, Strobl FJ. Journal of Clinical Apheresis 2003;18:51-54.

Thrombotic thrombocytopenic purpura (TTP) was once uniformly fatal. Therapeutic plasma exchange (TPE) in combination with immunosuppressive and anti-platelet agents, however, have resulted in improved survival rates of greater than 80% for patients with TTP. In spite of aggressive TPE and adjuvant therapy, a number of TTP patients are refractory to treatment. In addition, up to 40% of TTP patients who initially respond to therapy eventually relapse. Alternative therapies such as splenectomy have been used with varying degrees of success in refractory and relapsing TTP patients. The usefulness of splenectomy in preventing relapse of TTP or treating those patients who are refractory to TPE remains controversial. This paper presents a single institution's experience with 14 patients with nonfamilial TTP who underwent splenectomy for refractory (six patients) or relapsed (eight patients) TTP since 1984. The majority of patients (11) were female and the mean age was 42 years. In both patient groups, splenectomy induced stable long-term remissions. Six of six (100%) patients who were refractory to TPE survived to be discharged from the hospital, apparently free of disease. The average follow-up period was 147 months with no history of post-surgery relapse; 2 patients were lost-to-follow-up and one died several years later of unrelated causes. Four of eight patients (50%) who had a splenectomy for relapsing TTP went into a complete remission and had no further relapses of their disease. The average follow-up was 31 months. Moreover, in relapsing patients who failed to experience long-term remission, the relapse rate after splenectomy was 0.3 events per patient year compared to 1.0 events per patient year prior to splenectomy. Two relapsing patients died of causes unrelated to TTP and two died of acute TTP-related causes. The authors conclude that splenectomy is a reasonable treatment option for TTP patients refractory to standard TPE or who have experienced multiple and/or complicated relapses.