Perricone Carlo, Ceccarelli Fulvia, Nesher Gideon, Borella Elisabetta, Odeh Qasim, Conti Fabrizio, Shoenfeld Yehuda, Valesini Guido
Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Rome, Italy.
Immunol Res. 2014 Dec;60(2-3):226-35. doi: 10.1007/s12026-014-8597-x.
Immune thrombocytopenic purpura (ITP) is an autoimmune condition characterized by low platelet count with mucocutaneous and other bleedings. Clinical manifestations may range from spontaneous formation of purpura and petechiae, especially on the extremities, to epistaxis, bleeding at the gums or menorrhagia, any of which occur usually if the platelet count is below 20,000 per μl. A very low count may result in the spontaneous formation of hematomas in the mouth or on other mucous membranes. Fatal complications, including subarachnoid or intracerebral, lower gastrointestinal or other internal bleeding can arise due to an extremely low count. Vaccines may induce ITP by several mechanisms. Vaccine-associated autoimmunity may stem not only from the antigen-mediated responses but also from other constituents of the vaccine, such as yeast proteins, adjuvants, and preservatives diluents. The most likely is through virally induced molecular mimicry. The binding of pathogenic autoantibodies to platelet and megakaryocytes may cause thrombocytopenia by different mechanisms, such as opsonization, direct activation of complement, or apoptotic pathways. The autoantibodies hypothesis is not sufficient to explain all ITP cases: In the anti-platelet antibody-negative cases, a complementary mechanism based on T cell immune-mediated mechanism has been suggested. In particular, T cell subsets seem dysregulated with an increased production of pro-inflammatory cytokines, as IFN-γ and TNF, and chemokines, as CXCL10. Vaccines are one of the most striking discoveries in human history that changed dramatically life expectancy. Nonetheless, the occurrence of adverse events and autoimmune phenomena has been described following vaccination, and ITP may represent one of this.
免疫性血小板减少性紫癜(ITP)是一种自身免疫性疾病,其特征为血小板计数低,并伴有皮肤黏膜及其他部位出血。临床表现多样,从紫癜和瘀点的自发形成,尤其是在四肢,到鼻出血、牙龈出血或月经过多,通常当血小板计数低于每微升20,000个时就会出现上述任何一种情况。极低的计数可能导致口腔或其他黏膜自发形成血肿。由于计数极低,可能会出现致命并发症,包括蛛网膜下腔或脑内出血、下消化道或其他内出血。疫苗可通过多种机制诱发ITP。疫苗相关的自身免疫不仅可能源于抗原介导的反应,还可能源于疫苗的其他成分,如酵母蛋白、佐剂和防腐剂稀释剂。最可能的是通过病毒诱导的分子模拟。致病性自身抗体与血小板和巨核细胞的结合可能通过不同机制导致血小板减少,如调理作用、补体的直接激活或凋亡途径。自身抗体假说不足以解释所有ITP病例:在抗血小板抗体阴性的病例中,有人提出了基于T细胞免疫介导机制的互补机制。特别是,T细胞亚群似乎失调,促炎细胞因子如IFN-γ和TNF以及趋化因子如CXCL10的产生增加。疫苗是人类历史上最显著的发现之一,极大地改变了预期寿命。尽管如此,接种疫苗后仍有不良事件和自身免疫现象的报道,ITP可能就是其中之一。