We present a case of acute myeloid leukemia (AML) with myelodysplasia-related changes that presented as thrombotic thrombocytopenic purpura (TTP). Our patient presented with the classic pentad of TTP symptoms: anemia, thrombocytopenia, fever, elevated creatinine, and altered mental status. After a failure to respond to plasmapheresis therapy, we proceeded with a bone marrow biopsy and fluorescent in situ hybridization, which supported formal diagnosis of AML with myelodysplasia-related changes. Our case is an extremely rare presentation of a rare condition, as there have been no reported cases of AML with myelodysplasia-related changes presenting as TTP.

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Copyright © 2020, Kucharik et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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