Canberra Hospital, Canberra, ACT.
Australian National University, Canberra, ACT.
Med J Aust. 2022 Jan 17;216(1):43-52. doi: 10.5694/mja2.51284. Epub 2021 Oct 10.
The absence of high quality evidence for basic clinical dilemmas in immune thrombocytopenic purpura (ITP) underlines the need for contemporary guidelines relevant to the local treatment context. ITP is diagnosed by exclusions, with a hallmark laboratory finding of isolated thrombocytopenia.
Bleeding, family and medication histories and a review of historical investigations are required to gauge the bleeding risk and possible hereditary syndromes. Beyond the platelet count, the decision to treat is affected by individual bleeding risk, disease stage, side effects of treatment, concomitant medications, and patient preference. Treatment is aimed at achieving a platelet count > 20 × 10 /L, and avoidance of severe bleeding. Steroids are the standard first line treatment, with either 6-week courses of tapering prednisone or repeated courses of high dose dexamethasone providing equivalent efficacy. Intravenous immunoglobulin can be used periprocedurally or as first line therapy in combination with steroids.
There is no consensus on choice of second line treatments. Options with the most robust evidence include splenectomy, rituximab and thrombopoietin receptor agonists. Other therapies include azathioprine, mycophenolate mofetil, dapsone and vinca alkaloids. Given that up to one-third of patients achieve a satisfactory haemostatic response, splenectomy should be delayed for at least 12 months if possible. In life-threatening bleeding, we recommend platelet transfusions to achieve haemostasis, along with intravenous immunoglobulin and high dose steroids.
免疫性血小板减少症（ITP）基本临床困境缺乏高质量证据，这突显了制定与当地治疗环境相关的当代指南的必要性。ITP 通过排除法诊断，其特征性实验室发现是孤立性血小板减少症。
需要进行出血、家族史和用药史评估，并回顾既往检查结果，以评估出血风险和可能的遗传性综合征。除血小板计数外，治疗决策还受个体出血风险、疾病阶段、治疗副作用、合并用药和患者偏好的影响。治疗目的是使血小板计数>20×10/L，避免严重出血。皮质类固醇是标准的一线治疗药物，无论是 6 周疗程的泼尼松减量还是重复疗程的高剂量地塞米松，其疗效相当。静脉注射免疫球蛋白可在治疗过程中使用，也可与皮质类固醇联合作为一线治疗药物。
对于二线治疗药物的选择尚无共识。最有证据支持的选择包括脾切除术、利妥昔单抗和血小板生成素受体激动剂。其他治疗方法包括硫唑嘌呤、霉酚酸酯、达那唑和长春碱类药物。鉴于多达三分之一的患者获得了令人满意的止血反应，如果可能的话，应至少延迟 12 个月进行脾切除术。在危及生命的出血情况下，我们建议输注血小板以止血，并给予静脉注射免疫球蛋白和大剂量皮质类固醇。
