Tarantino M D, Goldsmith G
University of Louisville School of Medicine, KY 40202, USA.
Semin Hematol. 1998 Jan;35(1 Suppl 1):28-35.
Medical history, physical examination, and laboratory testing are essential to arriving at the diagnosis of acute immune thrombocytopenic purpura (ITP). A history of recent viral illness occurs in about half of the pediatric patients who present with acute symptoms of ITP. The physical examination is normal except for purpura; a complete blood cell count with a differential white blood cell count can be used to confirm the diagnosis of acute ITP. Treatment decisions for acute ITP remain controversial. Treatment generally is designed to prevent life-threatening complications, such as intracranial hemorrhage, and may include single or combination therapy with corticosteroids, intravenous immunoglobulin (IVIg), anti-D, and splenectomy. Corticosteroids are inexpensive and offer an alluring option, especially in the recent era of cost-containment. The often slow platelet response and the potentially severe adverse effects of corticosteroid therapy are frequently a deterrent. IVIg usually leads to a rapid rise in platelet count; however, IVIg is very expensive and adverse effects associated with its infusion are common and sometimes troublesome. The role of anti-D in acute ITP is still evolving. It is similar to IVIg in platelet response and is considerably less expensive. Some degree of hemolysis, the main adverse reaction with anti-D, is inevitable due to the binding of anti-D antibody to Rh-positive erythrocytes. However, most cases of hemolysis do not require medical intervention. Splenectomy is reserved for refractory thrombocytopenia with life-threatening hemorrhage in acute ITP or after recurrent severe thrombocytopenia in chronic ITP. Other immunomodulatory therapies are also discussed.
病史、体格检查和实验室检查对于急性免疫性血小板减少性紫癜(ITP)的诊断至关重要。约一半出现ITP急性症状的儿科患者有近期病毒感染病史。除紫癜外,体格检查通常正常;全血细胞计数及白细胞分类计数可用于确诊急性ITP。急性ITP的治疗决策仍存在争议。治疗通常旨在预防危及生命的并发症,如颅内出血,可能包括单用或联合使用皮质类固醇、静脉注射免疫球蛋白(IVIg)、抗-D和脾切除术。皮质类固醇价格低廉,是一个有吸引力的选择,尤其是在当前成本控制的时代。皮质类固醇治疗的血小板反应通常较慢,且可能有严重的不良反应,这常常是一个阻碍因素。IVIg通常会使血小板计数迅速上升;然而,IVIg非常昂贵,其输注相关的不良反应很常见,有时还很麻烦。抗-D在急性ITP中的作用仍在演变。它在血小板反应方面与IVIg相似,且成本要低得多。由于抗-D抗体与Rh阳性红细胞结合,某种程度的溶血作为抗-D的主要不良反应是不可避免的。然而,大多数溶血病例不需要医学干预。脾切除术适用于急性ITP中伴有危及生命出血的难治性血小板减少症或慢性ITP反复出现严重血小板减少症之后的情况。还讨论了其他免疫调节疗法。