Naithani Rahul, Agrawal Neerja, Mahapatra M, Kumar Rajat, Pati H P, Choudhry V P
Pediatr Hematol Oncol. 2007 Jun;24(4):309-15. doi: 10.1080/08880010701360783.
The clinical and hematological profile and treatment outcome of children with warm autoimmune hemolytic anemia (AIHA) were assessed using retrospective case record analysis. There were 26 (17 idiopathic; 9 secondary) patients with a median age of 11 years. Pallor (100%), fever (39%), and jaundice (59%) were the main presenting complaints. Jaundice was much more common in idiopathic (70%) compared to secondary (44%). Direct antiglobulin test was negative in 3 patients. Oral prednisolone produced remission in 81% patients. Four patients relapsed after a median period of 7 months (2 months to 2 year) after response. All responded to a second course of steroids in median 14 days. One child required cyclosporin A in addition. No correlation was found between response and parameters such as age, sex, jaundice, low pretreatment hemoglobin, reticulocyte count, total leukocyte count, platelet count, subtype of AIHA, and hepatosplenomegaly. Relapse correlated with increased duration between the onset of symptoms and treatment. This study indicates that oral prednisolone is an effective therapy for autoimmune hemolytic anemia. In refractory cases cyclosporine A may be useful.
采用回顾性病例记录分析评估温抗体型自身免疫性溶血性贫血（AIHA）患儿的临床和血液学特征及治疗结果。共有26例患者（17例特发性；9例继发性），中位年龄为11岁。主要临床表现为面色苍白（100%）、发热（39%）和黄疸（59%）。与继发性AIHA（44%）相比，黄疸在特发性AIHA中更为常见（70%）。3例患者直接抗球蛋白试验阴性。口服泼尼松龙使81%的患者病情缓解。4例患者在缓解后中位7个月（2个月至2年）复发。所有患者在中位14天内对第二疗程的类固醇治疗均有反应。1例患儿还需要环孢素A治疗。未发现反应与年龄、性别、黄疸、治疗前低血红蛋白、网织红细胞计数、总白细胞计数、血小板计数、AIHA亚型及肝脾肿大等参数之间存在相关性。复发与症状出现至治疗之间的时间延长有关。本研究表明，口服泼尼松龙是自身免疫性溶血性贫血的有效治疗方法。在难治性病例中，环孢素A可能有用。
