Gupta V, Shukla J, Bhatia B D
Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, India.
Indian J Pediatr. 2008 May;75(5):451-4. doi: 10.1007/s12098-008-0071-0. Epub 2008 Jun 8.
To study the clinico-hematological profile and treatment outcome in children suffering from auto immune hemolytic anemia (AIHA).
Twelve children were diagnosed with auto immune hemolytic anemia over a period of four years. Direct antiglobulin test was positive in all the cases. Other causes of hemolytic anemia like thalassemia syndromes, hereditary spherocytosis, G6PD deficiency were excluded by appropriate tests. The children were followed up for 6 months to 4 years.
The age ranged from 7 mth to 9 yr with a mean age of 4.51 yr. All patients had pallor as the presenting complaint followed by splenomegaly (83.3%), jaundice (66.7%), fever (50%) and bleeding manifestations (16.7%). 9 patients had primary disease and 3 had secondary disease. Tubercular infection was seen in 2 patients with secondary disease. Jaundice was seen equally in both the groups. Oral prednisolone produced remission in 83.3% cases. 4 patients (3 in primary and one in secondary group) had relapse after initial response. All responded to a second course of steroids but had subsequent relapses and developed a chronic course.
Autoimmune hemolytic anemia is an uncommon cause of hemolytic anemia in children. Tubercular infection is an underlying pathology in cases of secondary autoimmune hemolytic anemia. Although oral steroids induce remission in most of the cases, relapses are common.
研究自身免疫性溶血性贫血（AIHA）患儿的临床血液学特征及治疗效果。
在四年时间里，12名儿童被诊断为自身免疫性溶血性贫血。所有病例直接抗球蛋白试验均为阳性。通过适当检查排除了其他溶血性贫血病因，如地中海贫血综合征、遗传性球形红细胞增多症、葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症。对这些儿童进行了6个月至4年的随访。
年龄范围为7个月至9岁，平均年龄4.51岁。所有患者均以面色苍白为主要症状，其次是脾肿大（83.3%）、黄疸（66.7%）、发热（50%）和出血表现（16.7%）。9例患者为原发性疾病，3例为继发性疾病。2例继发性疾病患者出现结核感染。两组黄疸发生率相同。口服泼尼松龙使83.3%的病例缓解。4例患者（原发性3例，继发性1例）在初始缓解后复发。所有患者对第二疗程的类固醇治疗均有反应，但随后复发并发展为慢性病程。
自身免疫性溶血性贫血是儿童溶血性贫血的少见病因。结核感染是继发性自身免疫性溶血性贫血病例的潜在病理因素。虽然口服类固醇在大多数病例中可诱导缓解，但复发很常见。
