一名患有Fc（γ）RIIIb缺乏症的母亲体内因抗Fc（γ）RIIIb抗体导致的同种免疫性新生儿中性粒细胞减少症。

Isoimmune neonatal neutropenia due to anti-Fc(gamma) RIIIb antibody in a mother with an Fc(gamma) RIIIb deficiency.

作者信息

Maślanka K, Guz K, Uhrynowska M, Zupańska B

机构信息

Institute of Haematology & Blood Transfusion, Warsaw, Poland.

出版信息

Transfus Med. 2001 Apr;11(2):111-3. doi: 10.1046/j.1365-3148.2001.00294.x.

DOI:10.1046/j.1365-3148.2001.00294.x

PMID:11299028

Abstract

A rare case of neutropenia in a newborn due to anti-Fc(gamma) RIIIb antibody is described. The newborn, born from the 5th pregnancy, had severe infection and no neutrophils. Full clinical and neutrophil count recovery was observed when the child was 5 weeks old. In maternal serum, panreactive granulocyte alloantibodies were detected. The mother's and her two sisters' granulocytes appeared to be Fc(gamma) RIIIb deficient as found using pheno- and genotyping methods. All of them were healthy. The anti-Fc(gamma) RIIIb specificity of antibodies was identified by the monoclonal antibody immunobilization of neutrophil antigen assay. Such antibodies were not found in both sisters with the Fc(gamma) RIIIb deficiency, although they were pregnant, one of them on the seventh occasion.

摘要

本文描述了一例罕见的因抗Fc（γ）RIIIb抗体导致新生儿中性粒细胞减少症的病例。该新生儿为第5胎，出生时患有严重感染且无中性粒细胞。患儿5周龄时临床症状和中性粒细胞计数完全恢复。在母体血清中检测到全反应性粒细胞同种抗体。通过表型和基因分型方法发现，母亲及其两个姐妹的粒细胞似乎缺乏Fc（γ）RIIIb。她们所有人都很健康。通过中性粒细胞抗原单克隆抗体免疫固定试验确定了抗体的抗Fc（γ）RIIIb特异性。尽管Fc（γ）RIIIb缺乏的两个姐妹都怀孕了，其中一人是第7次怀孕，但她们体内均未发现此类抗体。