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一些持续性或慢性免疫性血小板减少症患者存在内在性血小板生成受损。

Intrinsically impaired platelet production in some patients with persistent or chronic immune thrombocytopenia.

作者信息

Rivière Étienne, Viallard Jean-François, Guy Alexandre, Kilani Badr, Vieira-Dias Juliana, Pons Anne C, Couffinhal Thierry, Pellegrin Jean-Luc, James Chloé

机构信息

Internal Medicine Department, Bordeaux University Hospital Centre, Pessac, France.

Adaptation Cardiovasculaire à l'Ischémie, University of Bordeaux, Pessac, France.

出版信息

Br J Haematol. 2015 Aug;170(3):408-15. doi: 10.1111/bjh.13444. Epub 2015 Apr 14.

Abstract

Persistent or chronic immune thrombocytopenias (P/C-ITP) are acquired blood disorders lasting more than 3 months or 1 year, respectively. The pathogenesis of these disorders is thought to be immunological. We hypothesized that some patients with P/C-ITP might have an intrinsic megakaryopoiesis defect. We identified a group of P/C-ITP patients with acquired isolated mild thrombocytopenia (30-100 × 10(9) /l), undetectable anti-platelet antibodies, negative autoimmune investigations and no need for treatment. We examined in vitro megakaryocyte differentiation and compared these patients' results with those of acute-ITP patients and healthy controls. No difference in proliferation, ploidy or expression of surface markers was found. In contrast, P/C-ITP patients had significantly fewer proplatelet-forming megakaryocytes. This novel observation demonstrated that some patients diagnosed with P/C-ITP have an intrinsic megakaryopoiesis defect independent of the bone-marrow environment. Further investigations are needed to dissect mechanisms underlying this impaired proplatelet formation in these patients.

摘要

持续性或慢性免疫性血小板减少症(P/C-ITP)是分别持续超过3个月或1年的后天性血液疾病。这些疾病的发病机制被认为是免疫性的。我们推测一些P/C-ITP患者可能存在内在的巨核细胞生成缺陷。我们识别出一组患有后天性孤立性轻度血小板减少症(30 - 100×10⁹ /l)、检测不到抗血小板抗体、自身免疫检查阴性且无需治疗的P/C-ITP患者。我们检测了体外巨核细胞分化情况,并将这些患者的结果与急性ITP患者和健康对照者的结果进行比较。在增殖、倍性或表面标志物表达方面未发现差异。相比之下,P/C-ITP患者形成前血小板的巨核细胞明显较少。这一新颖的观察结果表明,一些被诊断为P/C-ITP的患者存在独立于骨髓环境的内在巨核细胞生成缺陷。需要进一步研究以剖析这些患者中前血小板形成受损的潜在机制。

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