Chrobák L
II. interní klinika-oddĕlení klinické hematologie FN, Hradec Králové.
Vnitr Lek. 2002 Aug;48(8):773-80.
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder resulting from the binding of antibodies directed toward platelet surface glycoproteins GP IIb/IIIa and GP Ib/IX leading to their clearance by reticuloendothelial system. The diagnosis of ITP is made clinically by exclusion of other causes of thrombocytopenia. The treatment of chronic ITP is palliative, not curative and is directed toward inactivation and removal of a major site of platelet destruction and antibody production represented by the spleen. Spontaneous remission of ITP in adults are very rare. The goal of treatment for ITP is to prevent serious bleeding. About 30% of the affected patients show a long term response to steroid therapy. Splenectomy is the treatment of choice in the remaining patients. The treatment of patients refractory to corticosteroid therapy and splenectomy remains largely empirical and to date a generally accepted therapy has not been established. The newest approch to the treatment involves the use of monoclonal antibodies. In intracranial bleeding and in severe bleeding in refractory ITP use of rVIIa has been shown to might be useful. ITP in pregnancy represent a special situation. There is the trend toward treating these patients in a more conservative fashion.
特发性血小板减少性紫癜（ITP）是一种自身免疫性疾病，由针对血小板表面糖蛋白GP IIb/IIIa和GP Ib/IX的抗体结合所致，导致这些血小板被网状内皮系统清除。ITP的诊断是通过临床排除其他血小板减少的原因来做出的。慢性ITP的治疗是姑息性的，而非治愈性的，其目的是使以脾脏为代表的血小板破坏和抗体产生的主要部位失活并将其去除。成人ITP的自发缓解非常罕见。ITP的治疗目标是预防严重出血。约30%的受影响患者对类固醇治疗有长期反应。脾切除术是其余患者的首选治疗方法。对皮质类固醇治疗和脾切除术难治的患者的治疗在很大程度上仍基于经验，迄今为止尚未确立一种普遍接受的治疗方法。最新的治疗方法涉及使用单克隆抗体。在颅内出血和难治性ITP的严重出血中，已证明使用重组活化凝血因子VII（rVIIa）可能有用。妊娠合并ITP代表一种特殊情况。目前有以更保守方式治疗这些患者的趋势。
