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Thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) are rare hematologic conditions and have high mortality. Both TTP and HLH result from deregulation of the immune system. There are no published reports of coexisting TTP and HLH in elderly patients.A 67-year-old Asian male presented with altered consciousness and fever for 2 days. Physical examination revealed markedly pale, mild icterus with petechiae and purpura. Initially, TTP was recognized in this patient. Bone marrow studies are suggested for evaluating elderly patients to assess specific causes, especially infection and neoplasm.The TTP was diagnosed based on typical history-related symptoms and a specific laboratory result of very low ADAMTS13 level. The diagnosis of HLH was determined after detection of high levels of ferritin and lactase dehydrogenase, which were confirmed by the presence of hemophagocytosis in the bone marrow.Systemic corticosteroids and plasma exchange were initiated as specific treatment of the patient.The patient died in 3 weeks from ventilator-associated pneumonia.The HLH should be tested using bone marrow studies and specific laboratory tests in patients with TTP.