Lyall E G, Lucas G F, Eden O B
Department of Haematology, Royal Hospital for Sick Children, Edinburgh.
J Clin Pathol. 1992 May;45(5):431-4. doi: 10.1136/jcp.45.5.431.
Assessment of the clinical and haematological course of autoimmune neutropenia of infancy (ANI) in a defined childhood population in the south east of Scotland.
From January 1986 to February 1991 all children presenting with persistent neutropenia were examined serologically for evidence of antigranulocyte antibodies. The clinical course of those children found to have anti-granulocyte antibodies was then closely monitored.
During the study period five children had serologically confirmed ANI, giving an annual incidence of approximately 1/100,000 in this population. All of these cases followed the classic benign course of the condition. The presenting illnesses were mild, often with superficial skin sepsis and the initial absolute neutrophil count (ANC) ranged from 0.00-0.87 x 10(9)/l. All have remained well with no serious infections. Two children attained a normal ANC after 14 and 24 months respectively, the others currently remain neutropenic.
Autoimmune neutropenia of infancy is a condition which rests on a serological diagnosis. It follows a chronic benign course and all children eventually attain a normal ANC. The level of antigranulocyte antibody in the serum often begins to wane prior to improvement in the ANC and can give an indication of when recovery will begin to occur.
评估苏格兰东南部特定儿童群体中婴儿自身免疫性中性粒细胞减少症（ANI）的临床和血液学病程。
从1986年1月至1991年2月，对所有出现持续性中性粒细胞减少症的儿童进行血清学检查，以寻找抗粒细胞抗体的证据。然后密切监测那些被发现有抗粒细胞抗体的儿童的临床病程。
在研究期间，有5名儿童经血清学确诊为ANI，该人群的年发病率约为1/100,000。所有这些病例均遵循该疾病的典型良性病程。就诊时病情较轻，常伴有浅表皮肤脓毒症，初始绝对中性粒细胞计数（ANC）范围为0.00 - 0.87×10⁹/L。所有患儿均状况良好，无严重感染。两名儿童分别在14个月和24个月后ANC恢复正常，其他儿童目前仍为中性粒细胞减少症。
婴儿自身免疫性中性粒细胞减少症是一种基于血清学诊断的疾病。它遵循慢性良性病程，所有儿童最终ANC都会恢复正常。血清中抗粒细胞抗体水平通常在ANC改善之前开始下降，并可提示何时开始恢复。
