Refractory Primary Immune Thrombocytopenia With Bleeding and Thrombosis: A Case Report

BACKGROUND This report describes the case of a 50-year-old White male who presented with primary immune thrombocytopenia (ITP). He had a history of a previous episode of cerebral ischemia 1year before ITP onset, and a smoking habit. The ITP outcome was characterized by bleeding and thrombosis. Bleeding caused by low platelets is a characteristic of ITP, along with refractoriness to treatments and thrombosis caused by young activated platelets and treatments. CASE REPORT We report a 50-year-old man who presented with ITP and severe thrombocytopenia (<30×10⁹/L). The presentation of this case was complex and challenging because the patient had a greater risk of bleeding related to positive antiplatelet antibodies and a greater risk of thrombosis related to smoking and previous cerebral ischemia. He was nonresponsive to initial prednisone treatment and had petechial gastritis. He refused further oral corticosteroid therapy. Still, the patient was refractory to other therapeutic options, including vincristine and anti-D immunoglobulins, showing only transient platelet count response while also showing cerebral hemorrhage. Hence, he was treated with splenectomy and rituximab. This treatment, however, showed only an initial partial response and then no response. Therefore, he was started on romiplostim, a thrombopoietic receptor agonist, but this was stopped due to myocardial ischemia requiring dual antiplatelet therapy. A course of immunosuppressant therapy with azathioprine and mycophenolate mofetil was started with no response. Alternative therapies including eltrombopag, bortezomib, and dapsone resulted in no response, and romiplostim was re-started, causing acute coronary syndrome and unstable angina pectoris. Therefore, fostamatinib was started, but then was stopped due to melena. Finally, avatrombopag was started at a personalized dose of 20 mg daily in association with dual antiplatelet therapy, and this achieved good platelet count response. CONCLUSIONS This report shows that ITP is characterized by complexity, requiring management of bleeding/thrombotic risk using a tailored and patient-centric approach.