Prague Med. Rep. 2017, 118, 147-155

Acquired Amegakaryocytic Thrombocytopenic Purpura Progressing into Aplastic Anemia

Jan Philipp Novotný, Birgit Köhler, Regina Max, Gerlinde Egerer

Department of Medicine V, University of Heidelberg, Heidelberg, Germany

Received August 10, 2017
Accepted December 5, 2017

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare hematological disorder characterized by severe thrombocytopenia and a complete or near-to complete absence of megakaryocytes in the bone marrow, while granulopoiesis, as well as erythropoiesis are usually preserved. Although autoimmune mechanisms are believed to be causative, the exact underlying pathogenesis is not known. To date, only few cases have been reported and management of this disease remains controversial with immunosuppression being the treatment modality of choice in the majority of patients. In this article, we report a case of newly acquired AATP without an associated autoimmune disease, refractory to corticoids, intravenous immunoglobulin (IVIG) and second-generation TPO (thrombopoietin) agonists, which have recently been approved for the treatment of thrombocytopenia. Finally, in accordance with other reports, disease progression into aplastic anemia has occurred.


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