Durand Pauline, Pottier Valérie, Debordeaux Frédéric, Mesguich Charles, Duffau Pierre, Lazaro Estibaliz, Viallard Jean-François, Rivière Etienne
Department of Internal Medicine and Infectious Diseases, University Hospital Centre of Bordeaux, Pessac, France.
Department of Radiopharmacy, University Hospital Centre of Bordeaux, Pessac, France.
Br J Haematol. 2025 Jan;206(1):279-289. doi: 10.1111/bjh.19833. Epub 2024 Oct 15.
In primary immune thrombocytopenia (ITP), predictors of disease evolution and treatment response are needed. Data based on the site of platelet destruction are scarce. We performed a retrospective single-centre study of adult patients with primary ITP undergoing at least one Indium-111 platelet scintigraphy (IPS) between 2009 and 2018. Thirty-three patients had isolated hepatic platelet destruction (H-group), and 97 isolated splenic destruction (S-group). Median age at diagnosis (p < 0.001), proportion of associated cardiovascular (p < 0.001), organ-specific autoimmune diseases (p = 0.02), dependence on steroids (p = 0.003) and failure to rituximab (p = 0.01) were higher and relapse more frequent (p = 0.03) in H-group compared to non-splenectomized patients in S-group. Splenectomy was only performed in patients from S-group (as patients with hepatic sequestration are not splenectomized in our centre): 79% were in relapse-free remission at the end of a median 3.4-year post-IPS follow-up, 16% relapsed. In multivariate analyses, only a history of organ-specific autoimmune or inflammatory disease was significantly associated with hepatic sequestration (OR = 4.3, 95% CI = 1.2-15, p = 0.02). Patients with isolated hepatic sequestration were older, had more cardiovascular events and organ-specific autoimmune diseases, greater dependence on steroids, more relapses and a decreased response rate to rituximab suggesting an increased refractoriness to immunomodulatory therapies. Patients with isolated splenic sequestration responded well to splenectomy.
在原发性免疫性血小板减少症（ITP）中，需要了解疾病进展和治疗反应的预测因素。基于血小板破坏部位的数据很少。我们对2009年至2018年间至少接受过一次铟-111血小板闪烁显像（IPS）的成年原发性ITP患者进行了一项回顾性单中心研究。33例患者存在孤立性肝脏血小板破坏（H组），97例存在孤立性脾脏破坏（S组）。与S组未行脾切除术的患者相比，H组的诊断时中位年龄（p＜0.001）、合并心血管疾病的比例（p＜0.001）、器官特异性自身免疫性疾病的比例（p = 0.02）、对类固醇的依赖程度（p = 0.003）以及对利妥昔单抗治疗失败的比例（p = 0.01）更高，复发更频繁（p = 0.03）。仅对S组患者进行了脾切除术（因为在我们中心，肝脏扣押患者不行脾切除术）：在IPS后中位3.4年的随访结束时，79%的患者处于无复发缓解状态，16%的患者复发。在多变量分析中，只有器官特异性自身免疫或炎症性疾病史与肝脏扣押显著相关（OR = 4.3，95%CI = 1.2 - 15，p = 0.02）。存在孤立性肝脏扣押的患者年龄更大，有更多的心血管事件和器官特异性自身免疫性疾病，对类固醇的依赖性更强，复发更多，对利妥昔单抗的反应率降低，提示对免疫调节治疗的难治性增加。存在孤立性脾脏扣押的患者对脾切除术反应良好。
