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Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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Thrombotic thrombocytopenic purpura(TTP) is a rare, life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia caused by disseminated microvascular platelet-rich thrombi.The incidence of TTP is approximately 3.7 cases per 1,000,000 people.TTP is specifically associated with severe deficiency in ADAMTS13 activity. A study reported that among 772 patients with a first episode of TMA diagnosed as TTP, 166 patients(28%) had acquired TTP of unknown cause, defined as the absence of detectable anti-ADAMTS13 IgG and no ADAMTS13 mutations. Acquired TTP of unknown cause has been associated with a variety of factors, including idiopathic cases, autoimmune disease, infections, pregnancy, cancer, HIV, transplantation and drugs.
We present a rare case of an elderly patient who developed TTP without detectable anti-ADAMTS13 IgG, triggered by pneumonia, advanced age, infection, and multiple myeloma,along with a review of the literature.
Case:A 93-year-old man
Chief Complaint:Fever and coughPresent Illness:
A 93-year-old man developed fever 12 days before admission and was hospitalized with a diagnosis of aspiration pneumonia. On admission, his serum creatinine was 0.91 mg/dL and platelet count was 24,000/μL. The following day, schistocytes appeared on peripheral smear, renal function worsened (creatinine 1.10 mg/dL), and cerebral infarction developed. With a PLASMIC score of 6, thrombotic thrombocytopenic purpura (TTP) was suspected. Plasma exchange (PE) and three days of steroid pulse therapy were initiated, followed by oral prednisolone 50 mg/day. ADAMTS13 activity was subsequently found to be markedly reduced (<1%), confirming the diagnosis of TTP (ADAMTS13 inhibitor negative)
After five PE sessions, platelet count improved to 270,000/μL and PE was discontinued. However, platelet count later declined again. Since ADAMTS13 activity remained normal, TTP relapse was considered unlikely. Repeat PE showed minimal response, and platelet transfusions were given as supportive therapy. Bone marrow examination revealed multiple myeloma (MM) with monoclonal kappa light chain, which was considered to contribute to thrombocytopenia in addition to TTP.By the way, inhibitor and genetic abnormalities were negative.
We report a rare case of TTP triggered by pneumonia in an elderly patient, in whom MM was later diagnosed. A combination of factors—advanced age, infection, and multiple myeloma—may have contributed to decreased ADAMTS13 activity and the development of TTP.
