expert roundtables

Use of Splenectomy in the Treatment of Chronic Immune Thrombocytopenia

by Adam Cuker, MD, MS, Terry Gernsheimer, MD, Howard A. Liebman, MD

Overview

The use of splenectomy has declined in recent years with the introduction of additional medications for the treatment of patients with immune thrombocytopenia (ITP), such as rituximab and the thrombopoietin receptor agonists (TPO-RAs) romiplostim and eltrombopag. In patients with chronic ITP, platelet response is seen in 90% of those who undergo splenectomy, with 70% of patients remaining in remission. The response to splenectomy is higher than that seen with rituximab and is similar to that seen with long-term TPO-RA treatment. In addition to the efficacy reported with the TPO-RAs, the safety concerns and risk of mortality associated with splenectomy are also linked to the clinical decision to delay surgery in more patients. Patients failing first-line treatment with corticosteroids or intravenous immunoglobulin may be treated with a TPO-RA as a way to postpone surgery and assess whether splenectomy is actually necessary.

Q: Have recent treatment advances replaced splenectomy as the historic gold standard of treatment for patients with chronic ITP?

Adam Cuker, MD, MS

Assistant Professor of Medicine
Director, Penn Comprehensive Hemophilia and Thrombosis Program
Perelman School of Medicine
University of Pennsylvania
Philadelphia, PA

“Splenectomy will not go away completely, but it has moved farther down the list with the use of TPO-RAs, and it is the treatment of choice for fewer patients than it was at one time.”

Adam Cuker, MD, MS

Recent advances have absolutely changed the role of splenectomy in chronic ITP, and the proof is in the evidence that splenectomy is being used less frequently than it was historically. There are recently published institutional series where they compare their recent experience with their historical experience, and it is very clear that splenectomy rates have gone down as the use of medical therapies like rituximab and the TPO-RAs have gone up. However, there is an important place for splenectomy in the treatment of ITP. Splenectomy will not go away completely, but it has moved farther down the list with the use of TPO-RAs, and it is the treatment of choice for fewer patients than it was at one time.

Howard A. Liebman, MD

Donald I Feinstein Chair in Medicine
Professor of Medicine and Pathology
Jane Anne Nohl Division of Hematology
USC Norris Cancer Hospital
Los Angeles, CA

“With the use of TPO-RAs, splenectomy is not even up there as a second-line therapy anymore, and it has now become a third-line therapy.”

Howard A. Liebman, MD

I think that they have changed the algorithm about thinking about splenectomy. The historical algorithm is that after 6 months, you can take the patient off of steroids and think about splenectomy. This has changed because of moving the dividing line for chronic ITP out to a year, and also the advantages of other therapies have obviously lowered the use of splenectomy. Also, the Internet has played a big role in lowering the use of splenectomy because patients who always undergo a therapeutic intervention and fail that intervention, such as a surgical intervention, are really going to be very strong advocates against the use of surgery. This has happened with splenectomy, and, therefore, a lot of patients now come to the office with a body of information from the Internet, and one thing they do not want to have is surgery and splenectomy because the Internet says that splenectomy is not as efficacious. I inform them that there are some patients for whom splenectomy offers a chance of an unmaintained remission with the understanding that there are some long-term side effects with patients needing follow-up and immunizations. And that we give them an undated script at home for antibiotics any time they have a febrile illness, and we talk about other risks as if, again, the thrombotic risk that has emerged in long-term data. With the use of TPO-RAs, splenectomy is not even up there as a second-line therapy anymore, and it has now become a third-line therapy.

Terry B. Gernsheimer, MD

Director of the University of Washington
Medical Transfusion Service
Professor of Medicine, Division of Hematology
University of Washington
Seattle, WA

Splenectomy should be moved down in the guidelines, but it still may be used a lot. There are countries where you have to have a contraindication to splenectomy before they will allow you to get a TPO-RA, although I think that that is happening less and less.

References

Agnelli Giacchello J, Valeri F, Boccadoro M, Borchiellini A. Thrombopoietin receptor agonists in patients with persistent or chronic immune thrombocytopenia. Eur J Haematol. 2018;100(3):304-307.

Ahmed R, Devasia AJ, Viswabandya A, et al. Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children. Ann Hematol. 2016;95(9):1429-1434.

Chater C, Terriou L, Duhamel A, et al. Reemergence of splenectomy for ITP second-line treatment? Ann Surg. 2016;264(5):772-777.

Chaturvedi S, Arnold DM, McCrae KR. Splenectomy for immune thrombocytopenia: down but not out. Blood. 2018;131(11):1172-1182.

Kuter DJ, Bussel JB, Newland A, et al. Long-term treatment with romiplostim in patients with chronic immune thrombocytopenia: safety and efficacy. Br J Haematol. 2013;161(3):411-423.

Rodeghiero F. A critical appraisal of the evidence for the role of splenectomy in adults and children with ITP. Br J Haematol. 2018 Feb 26. doi: 10.1111/bjh.15090. [Epub ahead of print]

Saleh MN, Bussel JB, Cheng G, et al; EXTEND Study Group. Safety and efficacy of eltrombopag for treatment of chronic immune thrombocytopenia: results of the long-term, open-label EXTEND study. Blood. 2013;121(3):537-545.

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