Zeitschrift für Hämatologie und thromboembolische Erkrankungen
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Fatal Hematologic Alphabet Soup: A Complicated Case with Overlapping Features of TTP, AIHA, and HLH

Daniel B. Aruch, Louis Aledort

Here we describe the case of an unfortunate 26 year old woman who developed a fatal combination of autoimmune hemolytic anemia (AIHA), hemophagocytic lymphohistiocytosis (HLH), and thrombotic thrombocytopenic purpura (TTP). Her initial clinical picture looked that of typical autoimmune hemolytic anemia but when she was refractory to standard therapy and her course progressed, alternative diagnoses were evaluated despite the lack of schistocytes on her peripheral smear. The case highlights consideration of TTP in severe hemolysis in absence of schistocytes and investigation of HLH in severe multiorgan dysfunction even after a primary diagnosis, in this case AIHA, has been made.