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How I manage cyclic thrombocytopenia.

我如何处理周期性血小板减少症。

  • 影响因子:7.27
  • DOI:10.1182/blood.2020008218
  • 作者列表:"Kyrle PA","Eichinger S
  • 发表时间:2021-01-14
Abstract

:Cyclic thrombocytopenia (CTP) is a rare disease, which is characterized by periodic fluctuation of the platelet count. The pathogenesis of CTP is unknown and most likely heterogeneous. Patients with CTP are almost always misdiagnosed as having primary immune thrombocytopenia (ITP). The interval between ITP and CTP diagnosis can be many years. CTP patients often receive ITP-specific therapies including corticosteroids, thrombopoietin receptor agonists, rituximab, and splenectomy, which are followed by a transient increase in platelet count that is wrongly attributed to treatment effect with inevitable "relapse." CTP can be diagnosed by frequent platelet count monitoring, which reveals a typical pattern of periodic platelet cycling. An early diagnosis of CTP will prevent these patients from being exposed to possibly harmful therapies. The bleeding phenotype is usually mild and consists of mucocutaneous bleeding at the time when the platelet count is at its nadir. Severe bleeding from other sites can occur but is rare. Some patients respond to cyclosporine A or to danazol, but most patients do not respond to any therapy. CTP can be associated with hematological malignancies or disorders of the thyroid gland. Nevertheless, spontaneous remissions can occur, even after many years.

摘要

: 周期性血小板减少 (CTP) 是一种罕见疾病,其特征是血小板计数的周期性波动。CTP的发病机制是未知的,并且很可能是异质性的。CTP患者几乎总是被误诊为原发性免疫性血小板减少症 (ITP)。ITP和CTP诊断之间的间隔可以是多年。CTP患者通常接受ITP特异性治疗,包括皮质类固醇、血小板生成素受体激动剂、利妥昔单抗和脾切除术,随后血小板计数短暂增加,这被错误地归因于治疗效果,不可避免地 “复发”。CTP可以通过频繁的血小板计数监测来诊断,这揭示了周期性血小板循环的典型模式。CTP的早期诊断将防止这些患者暴露于可能有害的疗法。出血表型通常是轻度的,由血小板计数处于最低点时的皮肤黏膜出血组成。其他部位可发生严重出血,但罕见。一些患者对环孢素a或达那唑有反应,但大多数患者对任何治疗都没有反应。CTP可与血液恶性肿瘤或甲状腺疾病相关。然而,即使在许多年后,也可能发生自发缓解。

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