Pb2632: dillemas and challenges in itp treatment of a patient with thrombotic and hemorrhagic events development

HemaSphere(2023)

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摘要
Topic: 32. Platelet disorders Background: Immune thrombocytopenia (ITP) can paradoxically manifest with thrombosis, which can be explained by accelerated atherosclerosis. In active ITP, due to effect of rapid platelets displacement from bone marrow, level of circulating platelet micro-particle are increased, promoting thrombin formation. Up-to-date literature data reveals initial response to standard dose Rituximab in ITP treatment to be up to 64%, with complete response in half of cases. Unfortunately this treatment response decrease to 40% at two-year follow-up. Retreatment response is at 80%. Aims: Presenting a case of our patient diagnosed with ITP and multiple thrombotic events. Methods: We diagnosed our patient with ITP following laboratory, immunological, virological and radiographic (ultrasonography) diagnostics, including bonne marrow aspiration. Thrombotic events and subsequent hemorrhage were diagnosed via brain computerized tomography (CT), and acute coronary syndrome (ACS) was diagnosed by electrocardiogram, heart ultrasonography and cardiac enzyme findings. Therapy effect of ITP treatment was followed by regular blood count check-ups, and neurological status by control CT of brain. Results: Our male patient, aged 49, developed ACS in April 2022, and during cardiology examination subsequent blood analyses revealed low platelet count (plt), as low as 15x109/l. Following hematological diagnostics that included immunological, virological analysis, abdomen ultrasound and bone marrow aspiration, diagnose of primary ITP was established. Initial therapy option was introduction of Prednisone (1mg/kg/day) with partial remission effect (Plt maximum value was 57x109/l). A month after diagnose of ACS, and right after ITP diagnose, our patient developed right-sided hemiplegia and motor aphasia. Radiography (CT of brain) detected massive ischemic CVI which is possibly of cardio-embolic origin, especially that left ventricle hypokinesia was detected. Therefore, with careful and regular monitoring of platelet count, low molecular weight heparin (LMWH) was applied. Further neurological treatment and control radiography confirmed subacute CVI and right ACI occlusion (dissection). Also, control CT of brain confirmed development of consecutive CVI hemorrhagic transformation. Dexamethasone pulses were applied with peak of Plt recovery being 81x109/l. Because of trombogenic effect, intravenous immunoglobulin therapy was not applied. Following neurological stabilization, ITP treatment continued with Rituximab 375mg/m2 intravenous, in weekly regime (II of planned IV course, Plt peeking at 77x109/l). In further follow up control radiography confirmed regression of hemorrhage, with LMWH continuation. Summary/Conclusion: Rather challenging treatment of ITP patient with development of ACS, left ventricle hypokinesia, ischemic CVI with hemorrhagic transformation and right ACI dissection, requires very coordinated multidisciplinary approach with careful anticoagulant and questionable antiplatelet therapy application. This is further complicated due to risk of new bleeding and thrombotic events must also be taken in consideration. Furthermore, in,light of trombogenic potential of thrombopoietin receptor agonists, rituximab is most eligible therapy of choice. Keywords: Acute coronary syndrome, Idiopathic thombocytopenic purpura (ITP), Rituximab
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itp treatment,thrombotic,dillemas
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