Treatment of patients with refractory immune thrombocytopenia: literature review and case report

<p>ITP is a rare chronic autoimmune disease with isolated platelets decrease and high risk of bleeding complications. Standard treatment (steroids, HD of immunoglobulin and splenectomy) are effective in 70–90 % of patients, but in 10 % of them platelet count is not increased. A new group of drugs — TPO receptor agonists — is able to help to these patients. Their high efficacy in chronic ITP has shown in several studies, but the experience of their application before surgery is limited. We used romiplostim in 3 patients with chronic refractory ITP before surgery (2 — splenectomy and 1 — resection of nasal tumor). The<br />first patient, 19 years old, received multiple steroids, immunoglobulins and rituximab courses without effect during the last year. Platelets count was 7–15  10 9/l and hematuria and steroid tibia necrosis were revealed. Splenectomy was decided to be done. Because of the risk of hemorrhagic complications patients received romiplostim (3 mkg/kg) during 4 weeks. Upon reaching platelet counts 240  10 9/l splenectomy<br />was performed. A postoperative platelet count was 1200  10 9/l, 3 weeks later — 400  10 9/l. The second patient, 64 years old, with a<br />3-year ITP history was admitted to the hospital for splenectomy, but the platelet count (5.7  10 9/l) and a hemorrhagic syndrome with a constant need for platelet transfusions despite high doses of steroids and immunoglobulin received, interfere with the safety of operations.<br />Romiplostim was administered in increasing doses during 6 weeks to a maximum 10 mg/kg. Platelet count was 148  10 9/l and splenectomy was performed. Postoperative platelet count was 380  10 9/l, 3 weeks later — 120  10 9/l. The third patient, 22 years old, with a 15-year ITP history admitted with severe epistaxis. Nasal tumor was revealed. Patient was treated with immunoglobulins and steroids, and biopsy was attempt when platelet count increased to 50  10 9/l. This procedure ended with severe bleeding. Patient received 1 mg/kg of romiplostim and a week later platelet count was 250  10 9/l. Successfully tumor resection was done. No romiplostim side effects or thrombotic complications during postoperative period were found. The obtained data together with similar case reports of successful surgery after TPO-agonists administration allow considering romiplostim as an effective method of thrombocytopenia therapy before surgery in ITP patients.