Acquired Amegakaryocytic Thrombocytopenia in a Child: A Very Rare Case

Acquired amegakaryocytic thrombocytopenic purpuria (AATP) is an unusual hematologic disorder characterized by thrombocytopenia resulting from an unexplained reduction in the number of bone marrow megakaryocytes in the presence of otherwise normal hematopoiesis in the bone marrow [1]. The exact prevalence of acquired amegakaryocytic thrombocytopenia is unknown due to its very rare incidence. Around 30 cases are reported worldwide. Amegakaryocytic thrombocytopenic patients usually present with bleeding and thrombocytopenia not responding to treatment with steroids or IVIG. It is difficult to differentiate clinically and on peripheral blood findings from ITP. Bone marrow examination, particularly trephine biopsy is essential for this diagnosis. Based on the onset of presentation this can be classified as congenital or acquired. In congenital form (congenital AT) of manifestation, it is presented early and is associated with other features such as absent radii (TAR syndrome). In contrast the acquired type (Acquired AT) presents at any age and with prolonged thrombocytopenia [2].