PDF(Pubmed)
Abstract：
Thrombotic 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.
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.
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.
摘要：
背景：血栓性血小板减少性紫癜（TTP）是以血小板减少为特征的罕见且严重的血栓性微血管病，溶血性贫血,肾功能不全.相比之下,原发性血小板增多症（ET）是一种与血小板数量异常增加相关的骨髓增殖性疾病。先前的研究报道了TTP患者发生ET的几例。然而,ET患者并发TTP的病例以前没有报道.在这个案例研究中,我们介绍了一名TTP患者，该患者先前被诊断为ET。因此,据我们所知,这是ET中TTP的第一份报告。
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。
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。
