Ribeiro Rosângela de Albuquerque, Galiza Neto Gentil Claudino de, Furtado Amanda da Silva, Ribeiro Lucas Loiola Ponte Albuquerque, Kubrusly Marcela Sobreira, Kubrusly Elsie Sobreira
Hospital Universitário Walter Cantídio, Universidade Federal do Ceará (HUWC UFC), Fortaleza, CE, Brazil.
Hospital Universitário Walter Cantídio, Universidade Federal do Ceará (HUWC UFC), Fortaleza, CE, Brazil.
Hematol Transfus Cell Ther. 2019 Jul-Sep;41(3):253-261. doi: 10.1016/j.htct.2018.10.005. Epub 2019 Feb 16.
The management of adult (≥18 years) immune thrombocytopenia patients relies on platelet count, the risk of bleeding and presence of bleeding.
Confirming the diagnosis of immune thrombocytopenia and the start of therapy, our hematology service, a referral center, favors the establishment of this algorithm to treat those patients.
Presentation, recently diagnosed or recurrence - group 1: life-threatening bleeding: high-dose intravenous immunoglobulins with methylprednisolone or dexamethasone. Hospitalization and platelet transfusion are considered. Group 2: Platelets <30×10/L with bleeding or risk factor for bleeding, or platelets <20×10/L: prednisone or dexamethasone. No response, platelets <20×10/L: replace corticoid or increase doses. If platelets continue <20×10/L: immunization and splenectomy. Investigation of Helicobacter pylori, if positive: treatment for H. pylori. Chronic immune thrombocytopenia with platelets <20×10/L we propose two new groups (A and B): Group A: <65 years, no or low surgical risk, patient declines maintenance therapy or patient intends to get pregnant: immunization and splenectomy. Group B: failure of splenectomy (refractory) or no splenectomy indication or history of exposure to malaria or babesiosis and no response to corticoids or corticoid dependence: choose thrombopoietin receptor agonists: eltrombopag or romiplostim. Patient at high risk for arterial or venous thrombosis: recommend rituximab. After rituximab or thrombopoietin receptor agonists, if platelets continue <20×10/L: indicate immunosuppressants (azathioprine or cyclophosphamide), dapsone or mycophenolate mofetil or vinca alkaloids. The goals of treatment for chronic or refractory immune thrombocytopenia are to keep platelets >20×10/L and stop bleeding.
成人（≥18岁）免疫性血小板减少症患者的治疗取决于血小板计数、出血风险和出血情况。
作为转诊中心，我们的血液科为确诊免疫性血小板减少症并开始治疗，倾向于制定此算法来治疗这些患者。
初发、近期诊断或复发——第1组：危及生命的出血：大剂量静脉注射免疫球蛋白联合甲泼尼龙或地塞米松。考虑住院和输注血小板。第2组：血小板<30×10⁹/L且有出血或出血危险因素，或血小板<20×10⁹/L：泼尼松或地塞米松。无反应，血小板<20×10⁹/L：更换皮质类固醇或增加剂量。如果血小板持续<20×10⁹/L：免疫治疗和脾切除术。检测幽门螺杆菌，若阳性：治疗幽门螺杆菌。慢性免疫性血小板减少症且血小板<20×10⁹/L，我们提出两个新组（A组和B组）：A组：<65岁，手术风险低或无手术风险，患者拒绝维持治疗或患者打算怀孕：免疫治疗和脾切除术。B组：脾切除失败（难治性）或无脾切除指征或有疟疾或巴贝斯虫病暴露史且对皮质类固醇无反应或依赖皮质类固醇：选择血小板生成素受体激动剂：艾曲泊帕或罗米司亭。有动脉或静脉血栓形成高风险的患者：推荐利妥昔单抗。使用利妥昔单抗或血小板生成素受体激动剂后，如果血小板持续<20×10⁹/L：使用免疫抑制剂（硫唑嘌呤或环磷酰胺）、氨苯砜或霉酚酸酯或长春花生物碱。慢性或难治性免疫性血小板减少症的治疗目标是使血小板>20×10⁹/L并止血。
