Splenectomy for immune thrombocytopenia: down but not out

Abstract

word count: 239Word count: 4298 ABSTRACTSplenectomy is an effective therapy for steroid-refractory or dependent immune thrombocytopenia (ITP). With the advent of medical alternatives such as rituximab and thrombopoietin receptor antagonists (TPO-RA), the use of splenectomy has declined and is generally reserved for patients that fail multiple medical therapies. Splenectomy removes the primary site of platelet clearance and autoantibody production, and offers the highest rate of durable response (50-70%) compared to other ITP therapies. However, there are no reliable predictors of splenectomy response, and long-term risks of infection and cardiovascular complications must be considered. Since the long-term efficacy of different second-line medical therapies for ITP have not been directly compared, treatment decisions must be made without supportive evidence. Splenectomy continues to be a reasonable treatment option for many patients, including those with an active lifestyle who desire freedom from medication and monitoring, and patients with fulminant ITP that does not respond well to medical therapy. We try to avoid splenectomy within the first 12 months after ITP diagnosis for most patients to allow for spontaneous or therapy-induced remissions, particularly in older patients who have increased surgical morbidity and lower rates of response, and in young children. Treatment decisions must be individualized based on patients' comorbidities, lifestyles and preferences. Future research should focus on comparing long-term outcomes of patients treated with different second line therapies, and on developing personalized medicine approaches to identify subsets of patients most likely to respond to splenectomy or other therapeutic approaches.

Systematic literature review of treatments used for adult immune thrombocytopenia in the second‐line setting

“…Although not formally studied as a treatment arm in any prospective RCT, splenectomy is widely covered in the literature. Importantly, it is still generally recommended as the standard therapy for patients with chronic ITP, given the high probability of durable platelet response . However, this invasive surgical procedure is not without risk; perioperative and short‐term and long‐term postoperative complications, such as infections, thromboembolic events, and increased risk of certain malignancies including buccal, esophageal, colon, liver, pancreatic, lung, prostate, and hematopoietic cancers have been observed .…”

“…Splenectomy has historically been considered the second‐line therapy of choice in adult ITP . A systematic review was previously conducted to examine studies (published from 1966 to 2003) that assessed the efficacy/effectiveness of medical treatments for adult patients with ITP who have not responded to splenectomy.…”

Fostamatinib for the treatment of adult persistent and chronic immune thrombocytopenia: Results of two phase 3, randomized, placebo‐controlled trials

“…Splenectomy is the only potentially curative approach, which is effective in a majority of patients initially, but some patients subsequently relapse. This surgical option is declining in use due to the availability of noninvasive options . Medical therapies, such as rituximab, thrombopoietin receptor agonists, and immunosuppressive agents, have been effective in many patients and are chosen according to physician and patient preference.…”

A prospective, multicenter study of low dose decitabine in adult patients with refractory immune thrombocytopenia

“…Second, splenectomy is now frequently delayed with the increased preference on rituximab and thrombopoietin-receptor agonists. 9,10 If ITP patients do not respond to other therapies, splenectomy would be less likely to be effective in this setting. The IWG consensus report does not stipulate patient failure to multiple ITP-specific therapies or relapse.…”

“…It is undeniable that splenectomy continues to be a reasonable and effective treatment option for many patients, including those with an active lifestyle who desire freedom from medication and monitoring. 9,11 Decitabine is a hypomethylating agent with a dual mechanism of action. There is the demethylating effect leading to cell differentiation at low dose, and cytotoxic activity resulting in cell death at high concentration.…”