Romiplostim Before Splenectomy in a Patient with ITP - Case Report
In the following case report from authors at a university hospital in Barcelona, the successful use of romiplostim as a second-line treatment before planned splenectomy in a patient with immune thrombocytopenia (ITP), liver cirrhosis, and iatrogenic Cushing's syndrome is demonstrated.
Case Description
In 2002, a 64-year-old man was examined in the emergency room for rectal bleeding of unknown origin, simultaneously with severe thrombocytopenia detected in his blood count (13 × 109/L). Further examination revealed chronic liver disease, which doctors attributed to past alcohol consumption. The bone marrow showed sufficient megakaryocytes, excluding hematological causes of thrombocytopenia. However, thrombocytopenia persisted and was explained by splenomegaly associated with liver cirrhosis.
In the subsequent years, the patient experienced recurrent bleeding events, initially treated with intravenous immunoglobulins (IVIG) and prednisone with partial effect. In June 2009, he was re-hospitalized for ecchymoses, rectal bleeding, and recurrent severe thrombocytopenia. He received blood transfusions, high doses of corticosteroids, and IVIG. Repeated bone marrow aspirations found no abnormalities. After several more bleeding episodes with platelet counts of 1–3 × 109/L, long-term prednisone treatment was started at a dose of 100 mg daily. While this controlled the bleeding, the patient developed corticosteroid side effects, including Cushing's syndrome with cardiovascular complications (shortness of breath, edema, weight gain, blood pressure 180/90 mmHg).
It was then decided to move to the next line of ITP treatment with the aim of reducing corticosteroid doses. In March 2010, romiplostim, a thrombopoietin receptor agonist (TPO-RA), was introduced. Doctors set a safe platelet target range (50–90 × 109/L) to avoid increasing the risk of vascular thrombosis. After starting romiplostim in June 2010, dosage adjustments were necessary, but prednisone could be completely withdrawn by December 2010. In May 2011, with stable platelet levels, a laparotomy splenectomy was performed without hemorrhagic complications.
After 5 years of follow-up, the patient only requires vitamin supplements, has no bleeding episodes, his platelet count is within normal range, and liver functions are stable.
Conclusion
Romiplostim as a second-line treatment for ITP helped achieve stable platelet levels, allowing gradual withdrawal of corticosteroids, which were causing iatrogenic Cushing's syndrome, and prepared the patient for splenectomy. At the time of this case report's publication in 2017, recommendations for the treatment of adults with ITP who did not respond to initial IVIG or corticosteroids, or who relapsed during this treatment, included rituximab, TPO-RA (romiplostim or eltrombopag), or splenectomy as additional modalities.
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Source: Taltavuli T. C., Pena-Cala M. C. Romiplostim therapy as a second-line treatment before splenectomy for refractory immune thrombocytopenia in a cirrhotic patient with iatrogenic Cushing syndrome secondary to corticosteroids. Clin Case Rep 2017; 5 (2): 159–163, doi: 10.1002/ccr3.667.
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