Buchanan George R, Journeycake Janna M, Adix Leah
Division of Hematology-Oncology, Department of Pediatrics, The University of Texas Southwestern Medical Center at Dallas, Children's Medical Center of Dallas, Dallas, Texas, USA.
Semin Thromb Hemost. 2003 Dec;29(6):595-603. doi: 10.1055/s-2004-815627.
Chronic idiopathic thrombocytopenic purpura (ITP) can be categorized as mild, moderately severe, or severe. Severe chronic ITP during childhood is a rare disorder characterized by clinically significant mucocutaneous hemorrhage, usually in the setting of marked thrombocytopenia. It can cause substantial morbidity and rarely mortality. Many patients improve with time or even fully recover, but for those whose quality of life is negatively influenced by hemorrhage or side effects of conventional therapy (corticosteroids, intravenous immunoglobulin G, or anti-D), splenectomy is recommended. If splenectomy is unsuccessful or not feasible, other drug treatments are available, but few efficacy data exist, and the toxicity and cost of these treatments can be appreciable. Their use is best avoided outside of clinical trials conducted in specialty centers or in multi-institutional networks.
慢性特发性血小板减少性紫癜（ITP）可分为轻度、中度严重或重度。儿童期重度慢性ITP是一种罕见疾病，其特征为临床上显著的黏膜皮肤出血，通常发生在明显血小板减少的情况下。它可导致严重发病，很少导致死亡。许多患者会随时间改善甚至完全康复，但对于那些生活质量受到出血或传统治疗（皮质类固醇、静脉注射免疫球蛋白G或抗-D）副作用负面影响的患者，建议进行脾切除术。如果脾切除术不成功或不可行，可以采用其他药物治疗，但疗效数据很少，而且这些治疗的毒性和成本可能相当可观。最好避免在专科中心或多机构网络进行的临床试验之外使用这些治疗方法。
