Justiz Vaillant Angel A., Gupta Nagendra
University of the West Indies
Texas Health Resources
Immune thrombocytopenic purpura (ITP) is an autoimmune disease characterized by a low platelet count, purpura, and hemorrhagic episodes caused by antiplatelet autoantibodies. The diagnosis is typically made by excluding the known causes of thrombocytopenia. IgG autoantibodies sensitize the circulating platelets. It leads to the accelerated removal of these cells by antigen-presenting cells (macrophages) of the spleen and sometimes the liver or other components of the monocyte-macrophage system. The bone marrow compensates for platelet destruction by increasing platelet production. ITP most often occurs in healthy children and young adults within a few weeks following a viral infection. ITP is usually manageable with immunosuppressive therapy. An identical form of autoimmune thrombocytopenia can also be associated with chronic lymphocytic leukemia, lymphomas, SLE, infectious mononucleosis, and other bacterial and viral infections. Certain drugs can also cause immune thrombocytopenia indistinguishable from ITP. Most children have spontaneous remission within a few weeks or months, and splenectomy is rarely needed. However, young adults rarely have spontaneous remissions necessitating splenectomy within the first few months after diagnosis. The International Working Group on ITP defines ITP according to the following clinical phases. These are as follows: 1. Newly diagnosed ITP is in the first three months post-diagnosis. 2. Persistent ITP is for 3-12 months. 3. Chronic ITP is for > 12 months. 4. Refractory ITP is the failure of splenectomy.
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、紫癜以及由抗血小板自身抗体引起的出血发作。诊断通常通过排除已知的血小板减少原因来做出。IgG自身抗体使循环中的血小板致敏。这导致这些细胞被脾脏的抗原呈递细胞(巨噬细胞)加速清除,有时也会被肝脏或单核细胞 - 巨噬细胞系统的其他成分清除。骨髓通过增加血小板生成来补偿血小板的破坏。ITP最常发生在健康儿童和年轻成年人中,在病毒感染后的几周内。ITP通常通过免疫抑制疗法来控制。一种相同形式的自身免疫性血小板减少症也可能与慢性淋巴细胞白血病、淋巴瘤、系统性红斑狼疮、传染性单核细胞增多症以及其他细菌和病毒感染有关。某些药物也可引起与ITP难以区分的免疫性血小板减少症。大多数儿童在几周或几个月内会自发缓解,很少需要进行脾切除术。然而,年轻成年人很少有自发缓解,在诊断后的头几个月内需要进行脾切除术。国际ITP工作组根据以下临床阶段对ITP进行定义。具体如下:1. 新诊断的ITP是在诊断后的前三个月。2. 持续性ITP持续3 - 12个月。3. 慢性ITP持续超过12个月。4. 难治性ITP是脾切除术后失败的情况。