This case presents a rare instance of thrombotic thrombocytopenic purpura as an initial presentation of acute myeloblastic leukemia, highlighting the need to maintain a high degree of clinical suspicion for neoplastic causes in atypical thrombotic thrombocytopenic purpura. Although the association between acute myeloblastic leukemia and thrombotic thrombocytopenic purpura is anecdotal, the unusual clinical features, including atypical skin lesions in a 65-year-old patient with constitutional symptoms, led to the acute myeloblastic leukemia diagnosis. The pathophysiology may involve anti-ADAMTS13 antibodies or ADAMTS13 deficiency. Treatment is based on standard thrombotic thrombocytopenic purpura therapy (corticosteroids, plasmapheresis, and rituximab) while treating the underlying acute myeloblastic leukemia. A neoplasm, particularly a hematologic neoplasm, should be suspected in any elderly patient presenting with atypical thrombotic thrombocytopenic purpura. Given the poor prognosis of both entities separately, their association remains fatal.
Nassani et al. (2026) studied this question.
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