Mahévas M, Audia S, Viallard J-F
Service de médecine interne, centre de référence des cytopénies auto-immunes de l'adulte, centre hospitalier universitaire Henri-Mondor, Assistance Publique-Hôpitaux de Paris, université Paris-Est-Créteil, Créteil, France.
Service de médecine interne et immunologie clinique, médecine 1-SOC 1, hôpital François-Mitterrand, 14, rue Paul-Gaffarel, centre de référence des cytopénies auto-immunes de l'adulte, CHU de Dijon-Bourgogne, 21079 Dijon cedex, France.
Rev Med Interne. 2021 Jan;42(1):46-49. doi: 10.1016/j.revmed.2020.10.378. Epub 2020 Dec 15.
Multirefractory immune thrombocytopenia (ITP) is defined by the absence of response to TPO receptor agonists, rituximab and splenectomy (or contraindicated or refused) and the need of treatment. The approach to multirefractory ITP must be systematic and firstly involves reconsidering the diagnosis. Inherited thrombocytopenia, lymphoid hemopathies and myelodysplastic syndrome are the main causes to be mentioned. Multirefractory ITP is often associated with secondary ITP with signs of clinical or biological autoimmunity, monoclonal gammopathy of undetermined significance and a poor response to corticosteroids. Therapeutic management is complex and is based on the combination of treatments. New treatments are being developed.
难治性免疫性血小板减少症(ITP)的定义为对血小板生成素受体激动剂、利妥昔单抗和脾切除术无反应(或禁忌或拒绝)且需要治疗。难治性ITP的治疗方法必须系统,首先要重新考虑诊断。遗传性血小板减少症、淋巴造血系统疾病和骨髓增生异常综合征是主要应提及的病因。难治性ITP常与伴有临床或生物学自身免疫迹象、意义未明的单克隆丙种球蛋白病及对皮质类固醇反应不佳的继发性ITP相关。治疗管理复杂,基于多种治疗方法的联合。新的治疗方法正在研发中。