Thrombotic thrombocytopenic purpura in a patient on long-term alpha-interferon therapy for essential thrombocythemia: a case report

BMC Nephrology

Case Report

Dan Yin¹  Fang Liu²  Hongyu Qiu²  Chunmei Qin³ 

[1] Bioinformatics Under Biology Department, University of California-San Diego, San Diego, USA;Department of Nephrology, Kidney Research Institute, West China Hospital of Sichuan University, 37, Guoxue Alley, 610041, Chengdu, Sichuan Province, China;Department of Nephrology, Kidney Research Institute, West China Hospital of Sichuan University, 37, Guoxue Alley, 610041, Chengdu, Sichuan Province, China;Department of Nephrology, Luzhou People’s Hospital, Luzhou, China;

关键词: Essential thrombocythemia;    Thrombotic thrombocytopenic purpura;    Alpha interferon;    Plasma exchange;    Case report;   

DOI  :  10.1186/s12882-023-03200-7

 received in 2022-09-06, accepted in 2023-05-12,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundThrombotic thrombocytopenic purpura (TTP) is rare and severe thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and renal dysfunction. In contrast, essential thrombocythemia (ET) is a myeloproliferative disease associated with an abnormal increase in platelet numbers. Previous studies reported several cases of the development of ET in patients with TTP. However, the case of an ET patient complicated with TTP has not been previously reported. In this case study, we present a patient with TTP who was previously diagnosed with ET. Therefore, to the best of our knowledge, this is the first report of TTP in ET.Case presentationA 31-year-old Chinese female who was previously diagnosed with ET presented with anemia and renal dysfunction. The patient had been on long-term treatment with hydroxyurea, aspirin, and alpha interferon (INF-α) for ten years. The diagnosis of TTP was confirmed by clinical features, schistocytes noted on the peripheral blood smear, and lower ADAMTS13 activity (8.5%), together with the renal biopsy results. INF-α was discontinued, and the patient was then treated with plasma exchange and corticosteroids. After one year of follow-up, the patient had a normal hemoglobin level and platelet numbers, and her ADAMTS13 activity had improved. However, the patient’s renal function remains impaired.ConclusionsWe report a case of an ET patient complicated with TTP that was possibly due to INF-α, highlighting the potential complications associated with long-term ET therapy. The case also highlights the importance of considering TTP in patients with pre-existing ET who present with anemia and renal dysfunction, extending the spectrum of known studies.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

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Fig. 2