Idiopathic thrombocytopenic purpura (ITP) is a common immune disorder caused by platelet-reactive autoantibodies. Antibody-coated platelets are cleared more rapidly from the circulation, often in the spleen, than they can be replaced by compensatory stimulation of platelet production in the bone marrow. In some patients, platelet production is depressed as well. ITP in adults does not generally remit spontaneously, and most patients require treatment to prevent bleeding at one time or another. Therapy with corticosteroids, danazol, intravenous immune globulin, anti-D antibody, and several other agents inhibits clearance of the antibody-coated platelets but is rarely curative. Most patients will sustain a hemostatic response after splenectomy, although relapses may occur at any time. Patients may be more responsive to these same modalities after splenectomy, but treatment with an immunosuppressant that inhibits T- and B-cell function and cooperation, including azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, or anti-CD20, may be required. Antiviral therapy is useful in patients with HIV or hepatitis C infection, but no consensus has been reached as to the efficacy of antibiotics to eradicate Helicobacter pylori. Promising results have been seen in several patients treated with a modified thrombopoietin. It may be possible to design therapeutics that exploit the apparent restricted immunoglobulin gene usage by antiplatelet antibodies, perhaps in the form of engineered anti-idiotypic antibodies or other compounds that specifically target autoantibody-producing B cells. Rationale therapy awaits a more thorough understanding of autoantibody production.

译文

特发性血小板减少性紫癜 (ITP) 是由血小板反应性自身抗体引起的常见免疫紊乱。抗体包被的血小板从循环中 (通常在脾脏中) 的清除速度比通过骨髓中血小板产生的代偿性刺激代替的速度更快。在某些患者中,血小板的产生也会降低。成人的ITP通常不会自发汇出,大多数患者需要治疗以防止一次或另一次出血。皮质类固醇,达那唑,静脉免疫球蛋白,抗D抗体和其他几种药物的治疗可抑制抗体包被的血小板的清除,但很少治愈。尽管随时可能发生复发,但大多数患者在脾切除术后仍将维持止血反应。患者在脾切除术后可能对这些相同的方式更敏感,但可能需要使用抑制T细胞和b细胞功能及合作的免疫抑制剂治疗,包括硫唑嘌呤,环磷酰胺,环孢素,霉酚酸酯或anti-CD20。抗病毒治疗对HIV或丙型肝炎感染患者有用,但对于抗生素根除幽门螺杆菌的功效尚未达成共识。在用改良的血小板生成素治疗的几例患者中,已经看到了令人鼓舞的结果。可能有可能设计出通过抗血小板抗体利用明显受限的免疫球蛋白基因使用的疗法,也许以工程化抗独特型抗体或其他特异性靶向自身抗体产生b细胞的化合物的形式。基本原理疗法正在等待对自身抗体产生的更透彻的了解。

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