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澳大利亚原住民的同种免疫性新生儿中性粒细胞减少症:一种未被认识的疾病?

Alloimmune neonatal neutropenia in Australian aboriginals: an unrecognized disorder?

作者信息

Rodwell R L, Tudehope D I, O'Regan P, Minchinton R, Waters A H

机构信息

Department of Haematology, Mater Misericordiae Hospitals, Brisbane, Australia.

出版信息

Transfus Med. 1991 Mar;1(1):63-7. doi: 10.1111/j.1365-3148.1991.tb00011.x.

DOI:10.1111/j.1365-3148.1991.tb00011.x
PMID:9259829
Abstract

Alloimmune neonatal neutropenia (ANN) is reported for the first time in two Australian aboriginals. Both infants displayed the typical clinical features of ANN with profound neutropenia which persisted for 7 weeks and only minor infectious episodes. However, management strategies differed for the two infants because in one case (complicated by uncertain paternity) serological confirmation of ANN was not obtained until after recovery of the infant's neutrophil count. The maternal antibodies could not be assigned to known neutrophil antigen specificities and a new antigen may be involved. The antibodies were reactive with > 99% of neutrophils in a Caucasian population. Aboriginals comprise 1% of the total population of Australia and 1-2% of the obstetric population at our institution. Thus, ANN may be an unrecognized disorder in this ethnic group and a possible cause of neonatal infection and mortality.

摘要

首次在两名澳大利亚原住民中报告了同种免疫性新生儿中性粒细胞减少症(ANN)。两名婴儿均表现出ANN的典型临床特征,即严重中性粒细胞减少症持续7周,且仅有轻微感染发作。然而,两名婴儿的管理策略有所不同,因为在其中一例(因亲子关系不明而复杂化)中,直到婴儿中性粒细胞计数恢复后才获得ANN的血清学确诊。母体抗体无法归类为已知的中性粒细胞抗原特异性,可能涉及一种新抗原。这些抗体与高加索人群中>99%的中性粒细胞发生反应。原住民占澳大利亚总人口的1%,在我们机构的产科人群中占1 - 2%。因此,ANN在该种族群体中可能是一种未被认识的疾病,可能是新生儿感染和死亡的原因。

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