Liebman Howard
Hematology Section, Department of Medicine, University of Southern California-Keck School of Medicine, Division of Cancer Medicine and Blood Diseases, Los Angeles, CA.
Semin Hematol. 2007 Oct;44(4 Suppl 5):S24-34. doi: 10.1053/j.seminhematol.2007.11.004.
Immune thrombocytopenic purpura (ITP) can be classified as primary (known also as idiopathic thrombocytopenic purpura) or as secondary to an underlying condition such as a malignant or nonmalignant disorder. Commonly occurring conditions associated with secondary ITP include lymphoproliferative disorders (chronic lymphocytic leukemia [CLL], Hodgkin's disease and non-Hodgkin's lymphomas), autoimmune collagen vascular diseases (systemic lupus erythematosus [SLE], thyroid disease, antiphospholipid syndrome [APS]), and chronic infections (human immunodeficiency virus [HIV], Helicobacter pylori, hepatitis C virus [HCV]). The mechanism of platelet destruction in thrombocytopenias associated with lymphoproliferative disorders and collagen vascular diseases is identical to the autoimmune mechanism seen in primary ITP. Drug-induced thrombocytopenias are uncommon and generally resolve quickly upon drug discontinuation, but are often attributed to other causes. Platelet destruction in infection-associated ITP occurs via various mechanisms including accelerated platelet clearance due to immune complex disease as seen in HIV infection or cross-reactivity of anti-platelet glycoprotein antibodies and viral antigens in HIV, HCV, and H pylori infections (antigenic mimicry). In patients with HCV-related cirrhotic liver disease, splenic sequestration secondary to portal hypertension and decreased production of thrombopoietin may further contribute to development of thrombocytopenia. The current treatment paradigm for secondary ITP varies according to the underlying condition. Standard treatments for primary ITP (corticosteroids, IVIG, anti-D, splenectomy) are often successful in secondary ITP. In cases of ITP with H pylori and HCV infection, treatment should focus on the underlying disorder.
免疫性血小板减少性紫癜(ITP)可分为原发性(也称为特发性血小板减少性紫癜)或继发于潜在疾病,如恶性或非恶性疾病。与继发性ITP相关的常见疾病包括淋巴增殖性疾病(慢性淋巴细胞白血病[CLL]、霍奇金病和非霍奇金淋巴瘤)、自身免疫性胶原血管疾病(系统性红斑狼疮[SLE]、甲状腺疾病、抗磷脂综合征[APS])以及慢性感染(人类免疫缺陷病毒[HIV]、幽门螺杆菌、丙型肝炎病毒[HCV])。与淋巴增殖性疾病和胶原血管疾病相关的血小板减少症中血小板破坏的机制与原发性ITP中所见的自身免疫机制相同。药物性血小板减少症不常见,通常在停药后迅速缓解,但常被归因于其他原因。感染相关ITP中的血小板破坏通过多种机制发生,包括HIV感染中因免疫复合物疾病导致的血小板清除加速,或HIV、HCV和幽门螺杆菌感染中抗血小板糖蛋白抗体与病毒抗原的交叉反应(抗原模拟)。在丙型肝炎相关的肝硬化肝病患者中,门静脉高压继发的脾扣押和血小板生成素生成减少可能进一步导致血小板减少症的发生。继发性ITP的当前治疗模式因潜在疾病而异。原发性ITP的标准治疗(皮质类固醇、静脉注射免疫球蛋白、抗-D免疫球蛋白、脾切除术)在继发性ITP中通常也很成功。在伴有幽门螺杆菌和HCV感染的ITP病例中,治疗应侧重于潜在疾病。