Amegakaryocytic thrombocytopenia is a severe form of thrombocytopenia characterized by diminished or absent megakaryocytes in the bone marrow without other abnormalities. This condition manifests in both congenital and acquired forms. Congenital amegakaryocytic thrombocytopenia manifests with severe thrombocytopenia and bleeding issues at birth, often progressing to bone marrow failure, whereas acquired amegakaryocytic thrombocytopenia typically emerges later in life and may be linked to immune-mediated mechanisms.Diagnosis involves bone marrow biopsy and genetic testing. Clinicians diagnose congenital amegakaryocytic thrombocytopenia by assessing characteristic bone marrow features and confirming variations in the c-MPL gene. At the same time, diagnosing acquired amegakaryocytic thrombocytopenia relies on clinical suspicion and bone marrow examination showing reduced or absent megakaryocytes.Treatment approaches differ between the 2 forms. Allogeneic hematopoietic stem cell transplant is the sole curative option for the congenital form associated with a c-MPL mutation. At the same time, treating the acquired form involves addressing the underlying cause, often in conjunction with immunosuppressive medications or bone marrow transplantation. Management requires a comprehensive, multidisciplinary approach tailored to individual patient needs, focusing on bleeding manifestations and preventing progression to bone marrow failure. This topic discusses the etiology, pathophysiology, evaluation, and management of amegakaryocytic thrombocytopenia, providing healthcare professionals with the necessary knowledge to reduce overall morbidity and mortality associated with amegakaryocytic thrombocytopenia.
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