ABSTRACT We report a rare case of concomitant immune thrombotic thrombocytopenic purpura (iTTP) and Evans syndrome, complicated by multiple cerebral infarctions. The patient presented with severe thrombocytopenia and hemolytic anemia, as well as a positive direct Coombs test. The initial diagnosis was Evans syndrome. Although a negative direct Coombs test is typically characteristic of iTTP, the ADAMTS13 activity finding obtained on the fifth day of hospitalization confirmed the coexistence of iTTP. This case highlights the diagnostic challenge posed by overlapping autoimmune hematologic disorders and underscores the need to consider iTTP even in patients with a positive direct Coombs test, particularly when there are thrombotic complications such as cerebral infarctions. Awareness of such atypical presentations is essential because early recognition and prompt initiation of appropriate therapy can be lifesaving.
Ogawa et al. (Mon,) studied this question.