自身免疫性溶血性贫血在儿童中的临床表现和预后。
Autoimmune Hemolytic Anemia in Children: Clinical Profile and Outcome.
发表日期:2023 Feb 14
作者:
Shrutiprajna Kar, C G Delhi Kumar, Rakhee Kar, Abhishekh Basavarajegowda
来源:
INDIAN JOURNAL OF PEDIATRICS
摘要:
为了发现儿童AIHA的常见诱因、他们的临床情况、治疗反应和结果,进行了一项从2013年到2020年进行的前瞻性和回顾性的描述性研究。纳入了1个月至14岁的溶血性贫血和直接抗人球蛋白试验(DAT)阳性的儿童。将没有任何溶血临床实验室证据的阳性DAT儿童排除在外。数据通过结构化的信息表和临床实验室情况、治疗措施和疾病结果进行收集。共有46名1个月至14岁的儿童参与了该研究,发病年龄的平均值为8.7(±4.34)岁,其中24名(52.8%)为男性。29例(63%)出现了继发性病因,而17例(37%)发现了原发性病因。系统性红斑狼疮(SLE)是13例(45%)的常见触发因素,其次是恶性肿瘤的4例(14%)。苍白(98%)、肝肿大(72%)和脾肿大(48%)是最常见的临床表现。混合免疫表型在27例(59%)中观察到,温和型和冷凝集素型分别在12例(26%)和7例(15%)中观察到。所有儿童都接受了糖皮质激素治疗,而麦考酚酯常用作15例(33%)的二线治疗。13例(71%)原发性AIHA和仅4例(14%)继发性贫血达到完全缓解。总体而言,7名儿童(15%)死亡,所有人都属于继发性AIHA。本研究中继发性AIHA比原发性更常见,SLE是标准触发因素。原发性AIHA的预后比继发性AIHA更好。©2023。作者(们)在Dr.K C Chaudhuri基金会的独家许可下。
To discover the common triggers for AIHA in children, their clinical profile, treatment response, and outcome.This was an ambispective descriptive study conducted between 2013 and 2020. Children aged 1 mo to 14 y with hemolytic anemia and a positive direct antiglobulin test (DAT) were included. Children with a positive DAT but without any clinicolaboratory evidence of hemolysis were excluded. Data were collected from a structured pro forma with particulars comprising clinicolaboratory profile, treatment administered, and disease outcome.A total of 46 children (aged between 1 mo and 14 y) were enrolled in the study. The mean age of onset was 8.7 (± 4.34) y, and 24 (52.8%) were males. Secondary causes were observed in 29 (63%) cases, while the primary cause was found in 17 (37%). Systemic lupus erythematosus (SLE) was the common trigger in 13 (45%) cases, followed by malignancy in 4 (14%) cases. Pallor (98%), hepatomegaly (72%), and splenomegaly (48%) were the most commonly observed clinical signs. The mixed immunophenotype was observed in 27 (59%) cases, followed by warm type in 12 (26%) and cold agglutinin type in 7 (15%) cases. All children received glucocorticoid therapy, and mycophenolate mofetil was commonly used as second-line therapy in 15 (33%) cases. 13 cases (71%) of primary AIHA and only 4 (14%) cases of secondary anemia achieved complete remission. Overall, 7 children (15%) died, all belonging to secondary AIHA.Secondary AIHA was more common than primary in the present study, and SLE was the standard trigger. Primary AIHA carries a better prognosis than secondary.© 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.