Amegakaryocytic thrombocytopenia is a rare haematologic disorder characterised by a profound reduction in platelet production due to the near absence of megakaryocytes, leading to severe thrombocytopenia. This report elucidates the diagnostic and therapeutic challenges encountered in managing such rare haematological conditions alongside significant cardiovascular disease. We detail the case of a patient who presented with chest pain and was diagnosed with non-ST elevation myocardial infarction (NSTEMI). Subsequent investigations revealed severe thrombocytopenia and underlying triple vessel disease, complicating immediate surgical intervention. Initial management strategies aimed at treating presumed immune thrombocytopenia proved ineffective. A definitive diagnosis of amegakaryocytic thrombocytopenia was established following a bone marrow biopsy. Despite treatment adjustments, including the administration of thrombopoietin agonists and immunosuppression, platelet counts improved but did not reach levels safe for coronary artery bypass grafting. This case underscores the importance of diagnosis and treatment of systemic disorders prior treatment of cardiac disease. It also demonstrates the importance of interdisciplinary cooperation in the treatment of a complex patient case.
Learning points: Amegakaryocytic thrombocytopenia is a rare cause of thrombocytopenia that can co-occur with cardiac conditions.The diagnosis of amegakaryocytic thrombocytopenia can be difficult as it can be mistaken for immune mediated thrombocytopenia, but distinction is critical as treatments differ.Modern patient care frequently requires collaboration between different subspecialities.
Keywords: CABG; NSTEMI; amegakaryocytic thrombocytopenia; thrombocytopenia; triple vessel disease.
© EFIM 2024.