PRAGUE MEDICAL REPORT
PRAGUE MEDICAL REPORT
Prague Medical Report is an English quarterly published multidisciplinary biomedical journal. Prague Medical Report was founded as Sborník lékařský in May 1885. The journal presents public primary scientific publications, short communications, casuistry, and reviews. It contains articles based on important specialised lectures and symposia.

PRAGUE MEDICAL REPORT, Vol 118 No 4 (2017), 147–155

Acquired Amegakaryocytic Thrombocytopenic Purpura Progressing into Aplastic Anemia

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

DOI: https://doi.org/10.14712/23362936.2017.16
published online: 11. 01. 2018

abstract

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.

keywords: Acquired amegakaryocytic thrombocytopenic purpura; Pure megakaryocytic aplasia; TPO agonist; Aplastic anemia

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Acquired Amegakaryocytic Thrombocytopenic Purpura Progressing into Aplastic Anemia is licensed under a Creative Commons Attribution 4.0 International License.

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ISSN: 1214-6994
E-ISSN: 2336-2936

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