A 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.
We 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.
方法:一名31岁的中国女性,先前被诊断为ET,表现为贫血和肾功能不全。患者长期接受羟基脲治疗,阿司匹林,和α干扰素(INF-α)十年。TTP的诊断通过临床特征证实。外周血涂片上记录的分裂细胞,和较低的ADAMTS13活性(8.5%),以及肾活检结果。INF-α已停用,然后患者接受血浆置换和皮质类固醇治疗。经过一年的随访,患者的血红蛋白水平和血小板数量正常,她的ADAMTS13活性有所改善。然而,患者的肾功能仍然受损。
结论:我们报告一例ET患者并发TTP,可能是由于INF-α,强调与长期ET治疗相关的潜在并发症。该病例还强调了在存在贫血和肾功能不全的预先存在ET的患者中考虑TTP的重要性。扩展已知研究的范围。