Oliveira Maria Christina L A, Oliveira Benigna M, Murao Mitiko, Vieira Zilma Maria, Gresta Letícia T, Viana Marcos B
Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
J Pediatr (Rio J). 2006 Jan-Feb;82(1):58-62. doi: 10.2223/JPED.1438.
Autoimmune hemolytic anemia is characterized by the production of autoantibodies against erythrocyte membrane antigens. This study was carried out to identify the clinical, immunological and outcome characteristics of autoimmune hemolytic anemia patients treated at the (HC-UFMG) Pediatric Hematology Unit and the Hemocentro de Belo Horizonte.
We evaluated 17 patients younger than 15 years old admitted from 1988 to 2003 were evaluated. Autoimmune hemolytic anemia diagnosis was based on the presence of acquired hemolysis and confirmed by positive direct Coombs polyspecific test results. Clinical, laboratory, and outcome data were obtained from patient records.
The median age at diagnosis was 10.5 months. The direct Coombs polyspecific test was positive in 13 and negative in four patients. Monospecific testing was performed for 14 patients. The most frequent red cell autoantibody was IgG (five patients), followed by IgM in two. Thirteen patients had severe anemia and needed blood transfusions. Underlying diseases were identified in four patients: systemic lupus erythematosus, Hodgkin's lymphoma, autoimmune hepatitis and Langerhans cell histiocytosis. The remaining patients were classified as having primary disease. The median follow-up period was 11 months (5 to 23 months). Three children died, two after splenectomy and one with complications of the underlying disease.
Autoimmune hemolytic anemia is rare in children and adolescents. Although patients usually respond to corticosteroids and/or immunoglobulin, fatal cases can occur. Prognosis is worse in patients with chronic underlying diseases.
自身免疫性溶血性贫血的特征是产生针对红细胞膜抗原的自身抗体。本研究旨在确定在（HC-UFMG）儿科血液科和贝洛奥里藏特血液中心接受治疗的自身免疫性溶血性贫血患者的临床、免疫学和预后特征。
我们评估了1988年至2003年收治的17例15岁以下患者。自身免疫性溶血性贫血的诊断基于获得性溶血的存在，并通过直接抗人球蛋白多特异性试验结果阳性得到证实。临床、实验室和预后数据均从患者记录中获取。
诊断时的中位年龄为10.5个月。13例患者直接抗人球蛋白多特异性试验呈阳性，4例呈阴性。对14例患者进行了单特异性检测。最常见的红细胞自身抗体是IgG（5例患者），其次是IgM（2例）。13例患者患有严重贫血，需要输血。4例患者发现有基础疾病：系统性红斑狼疮、霍奇金淋巴瘤、自身免疫性肝炎和朗格汉斯细胞组织细胞增多症。其余患者被归类为原发性疾病。中位随访期为11个月（5至23个月）。3名儿童死亡，2例在脾切除术后死亡，1例死于基础疾病并发症。
自身免疫性溶血性贫血在儿童和青少年中较为罕见。尽管患者通常对皮质类固醇和/或免疫球蛋白有反应，但仍可能发生致命病例。患有慢性基础疾病的患者预后更差。
