Kategorie: Myeloproliferativní nemoci
Číslo abstraktu: E1418
Immune thrombocytopenia (ITP) is an acquired, immunologically determined disease characterized by a decrease in platelet count and the associated increased risk of bleeding. The spleen plays a major role in the pathogenesis of ITP, it is the site of antibody production and often the major site of antibody-mediated platelet destruction. Splenectomy was the first method for ITP therapy and has long been the standard second-line treatment for adults with ITP who do not respond to corticosteroids. Recent advances in the understanding of etiopathogenetic mechanisms of ITP led to the development of novel therapies and changed the role of splenectomy in the management of ITP.
Describe the population indicated for splenectomy (before and after new guidelines)
Evaluated efficacy and safety of splenectomy and therapeutic possibilities for refractory patients
We retrospectively analyzed the data of 98 patients with primary ITP from four Czech hematological centers who underwent splenectomy between years 1995 and 2014. The response was evaluated according to the ITP International Working Group guidelines.
In total, 98 patients (66% females) underwent splenectomy for ITP between 1995 and 2014 in four Czech hematological centers. The median age at diagnosis was 36 years (range 6-72) and median age at splenectomy was 38 years (range 17-73). 56% patients who were splenectomized before year 2010 had chronic ITP while 72% patients had chronic ITP if the splenectomy was performed after year 2010. Minority of patients had newly diagnosed ITP, in a subgroup of patients splenectomized before year 2010 it was 19% and in a subgroup of patients splenectomized after year 2010 were none with newly diagnosed ITP's. While the median interval between diagnosis and splenectomy in patients who were splenectomized before 2010 was 13 months, the median interval in patients who were splenectomized after 2010 was longer (32 months). After 2010, splenectomy was used most often as a third-line treatment. 25 patients in our group underwent labelled platelet scanning before splenectomy, majority of them had splenic uptake. The median platelet count before splenectomy was 65 x 109/l (range 1-330 x 109/l). 77 patients (79%) were prophylactic vaccinated against encapsulated bacterial pathogens. 61% of patients underwent splenectomy with an open technique and 39% with a laparoscopic technique and the rate of laparoscopic splenectomies increased over time. Of the 98 patients, 10 (11%) achieved a response (R) and 83 (84%) a complete response (CR), the overall response rate was 95%. 28 (29%) patients relapsed after a median time 6 months (range 1-125). 8 patients (8%) experienced peri-operative and 3 patients (3%) post-splenectomy complications. In 33 patients (34%) splenectomy was not successful because of the relapse or lack of response, but new treatment options especially TPO mimetics improved the outcome of these refractory patients.
Splenectomy is an effective treatment for ITP with two thirds of patients achieving long-term response and still has a place in the management of ITP. Recent trend is to postpone splenectomy until the chronic phase of the disease.
Keyword(s): Immune thrombocytopenia (ITP), Splenectomy
Datum přednesení příspěvku: 12. 6. 2015