Surgical Pathology and Cytopathology Unit, Department of Medicine-DIMED, University of Padova, Padova, Italy.
3rd Surgical Clinic, Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, Italy.
Eur J Haematol. 2021 Feb;106(2):281-289. doi: 10.1111/ejh.13547. Epub 2020 Dec 2.
Immune thrombocytopenia (ITP) is an acquired disorder, characterized by immune-mediated platelet destruction. The spleen plays a key pathogenic role in ITP and splenectomy is a valuable second-line therapy for this disease. Little is known on ITP spleen histology and response to splenectomy is unpredictable. This study aims to characterize ITP spleen histology and assess possible predictors of splenectomy outcome.
A series of 23 ITP spleens were retrospectively assessed for the following histological parameters: density of lymphoid follicles (LFs), marginal zones (MZs), T helper and cytotoxic T cells; presence of reactive germinal centers (GCs); width of perivascular T cell sheaths; and red pulp features. Clinical and histological data were matched with postsplenectomy platelet counts to assess their prognostic relevance.
Three histological patterns were documented: a hyperplastic white pulp pattern, a non-activated white pulp pattern (lacking GCs), and a white pulp-depleted pattern. Poor surgical responses were associated with presplenectomy high-dose steroid administration, autoimmune comorbidities and low T follicular helper cell density. The combination of such parameters stratified patients into different splenectomy response groups. The removal of accessory spleens was also associated with better outcome.
ITP spleens are histologically heterogeneous and clinical-pathological parameters may help predict the splenectomy outcome.
免疫性血小板减少症（ITP）是一种获得性疾病，其特征为免疫介导的血小板破坏。脾脏在 ITP 中起关键的致病作用，脾切除术是治疗该病的有价值的二线疗法。对于 ITP 脾脏的组织学了解甚少，且对脾切除术的反应难以预测。本研究旨在描述 ITP 脾脏的组织学特征，并评估脾切除术结果的可能预测因素。
回顾性评估了 23 例 ITP 脾脏的以下组织学参数：淋巴滤泡（LFs）、边缘区（MZs）、辅助性 T 细胞和细胞毒性 T 细胞的密度；反应性生发中心（GCs）的存在；血管周围 T 细胞鞘的宽度；以及红髓特征。将临床和组织学数据与脾切除术后血小板计数相匹配，以评估其预后相关性。
记录了三种组织学模式：增生性白髓模式、非激活的白髓模式（缺乏 GCs）和白髓耗竭模式。术前高剂量类固醇治疗、自身免疫合并症和滤泡辅助性 T 细胞密度低与手术反应不佳相关。这些参数的组合将患者分层为不同的脾切除术反应组。附加脾脏的切除也与更好的结果相关。
ITP 脾脏在组织学上是异质的，临床病理参数可能有助于预测脾切除术的结果。
