Chintagumpala M M, Steuber C P, Mahoney D H, Ogden A K, Fernbach D J
Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
Am J Pediatr Hematol Oncol. 1991 Spring;13(1):52-6. doi: 10.1097/00043426-199121000-00013.
Essential thrombocythemia (ET) is a rare disorder in children. An 11-year-old white boy was first seen in January 1986 with symptoms of abdominal pain. His platelet count was 1.5 million/mm3. Other hematological values and coagulation studies, including bleeding time, were normal. There was laboratory evidence of mild platelet dysfunction. Using the criteria of the Polycythemia Vera Study Group, a diagnosis of ET was made. He developed frequent headaches. Aspirin was prescribed for the next 2 years at varying doses and frequency. During the period, platelet counts ranged between 1 and 3 million/mm3. In view of progressive headaches and evidence of increasing platelet dysfunction, further treatment was indicated. The use of a new agent, anagrelide, reported effective in adults with ET, resulted in amelioration of symptoms and improvement in quantitative and qualitative platelet control with no significant untoward effects.
原发性血小板增多症(ET)在儿童中是一种罕见的疾病。一名11岁的白人男孩于1986年1月首次就诊,有腹痛症状。他的血小板计数为150万/mm³。其他血液学指标和凝血研究,包括出血时间,均正常。有实验室证据表明存在轻度血小板功能障碍。根据真性红细胞增多症研究组的标准,诊断为ET。他出现了频繁头痛。在接下来的2年里,根据不同剂量和频率开具了阿司匹林。在此期间,血小板计数在100万至300万/mm³之间。鉴于进行性头痛和血小板功能障碍加重的证据,需要进一步治疗。使用一种新药物阿那格雷,据报道对成人ET有效,症状得到改善,血小板数量和质量控制得到改善,且无明显不良反应。