Balancing Therapy with Thrombopoietin Receptor Agonists and Splenectomy in Refractory Immune Thrombocytopenic Purpura: A Case of Postsplenectomy Thrombocytosis Requiring Plateletpheresis

Abstract

Immune thrombocytopenic purpura (ITP) causes thrombocytopenia through the autoimmune destruction of platelets. Corticosteroids remain the first line of therapy, and traditionally splenectomy has been the second. While the availability of thrombopoietin receptor agonists (TPO-RAs) has expanded treatment options, there is little data for the ideal management of these agents in preparation for splenectomy. Thrombocytosis has been reported after splenectomy in patients treated with TPO-RA preoperatively, with one prior case requiring plateletpheresis for symptomatic thrombocytosis. We present a case report and review of the literature pertaining to this complication and provide recommendations for preventing postsplenectomy thrombocytosis in ITP patients on TPO-RAs.

A Case of Primary Refractory Immune Thrombocytopenia: Challenges in Choice of Therapies

“…It is possible that the postsplenectomy status made our patient more susceptible to thrombocytosis. Severe postsplenectomy thrombocytosis requiring plateletpheresis has been reported in patients receiving romiplostim preoperatively [ 17 , 18 ], but unlike our case, these patients suffered from thrombocytosis in the immediate postoperative period. When rituximab and romiplostim are used as single therapy, splenectomy does not influence their response rate [ 13 , 19 ].…”

Perioperative oral eltrombopag versus intravenous immunoglobulin in patients with immune thrombocytopenia: a non-inferiority, multicentre, randomised trial

“…26,27 Use of thrombopoietin receptor agonists around the time of represented a unique clinical challenge owing to the risk of postsplenectomy thrombocytosis, which has been reported previously. 28 We observed two patients on eltrombopag who developed platelet counts above 1000 × 10⁹ cells per L after splenectomy. Similarly, rebound thrombocytopenia was observed in two patients after eltrombopag was stopped.…”

Short-term dose-escalated romiplostim for preparing an adult patient with persistent newly diagnosed primary immune thrombocytopenia for splenectomy

“… 8 Using a higher initial dose, the time to achieve >30,000 platelets/mm 3 was shorter and the response rate higher. 9 Management of refractory ITP remains a challenge and the optimal sequence of treatment is not known, nor is the role of the combination therapy well defined. After failing second-line therapy, the use of a TPO-RA at an escalated dose was proposed.…”

“…Reactive post-splenectomy thrombocytosis is reported in approximately 80% of the cases and may reach extreme levels in some 20% of the patients. 9 The use of TPO-RAs is also reported to be associated with thrombosis due to thrombocytosis. 10 In this report, the platelet count reached 1,300,000 platelets/mm 3 ten days post-surgery.…”

“…Thrombo-prophylaxis was started 24 h after surgery, with a combination of low molecular weight heparin and aspirin 100 mg/day, as has been suggested. 9 …”