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抗 HLA A31 和 B61 抗体导致的新生儿同种免疫性血小板减少症合并唐氏综合征。

Down syndrome with neonatal alloimmune thrombocytopenia due to anti-HLA A31 and B61 antibodies.

机构信息

Department of Pediatrics, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, Fukushima, 960-1295, Japan.

Department of Pediatrics, Ohta Nishinouchi Hospital, Koriyama, Japan.

出版信息

Int J Hematol. 2021 Jun;113(6):945-949. doi: 10.1007/s12185-021-03092-z. Epub 2021 Feb 11.

Abstract

Neonatal alloimmune thrombocytopenia (NAIT) arises from fetomaternal platelet incompatibility that results in transplacental passage of maternal antibodies mostly against fetal human platelet antigens (HPA), whereas NAIT due to anti-human leukocyte antigen (HLA) antibodies is extremely rare. Here, we report a case of Down syndrome (DS) with NAIT that was attributed to HLA antibodies. A boy with DS was delivered at 36 weeks' gestation. His platelet count declined to 13.0 × 10/L, suggestive of NAIT rather than other conditions, including transient abnormal myelopoiesis. Random platelet concentrates and intravenous immunoglobulin administration resolved the thrombocytopenia without clinical complications. Immunoserological investigations detected anti-HLA, but no anti-HPA antibodies in samples from the patient and the mother. HLA typing and cross-matching indicated that anti-HLA antibodies to paternal HLA A31 and B61, which had probably been induced during a prior pregnancy, led to NAIT in this case. Although it is a rare condition, healthcare providers should consider NAIT due to HLA antibodies and be vigilant for subsequent cases in DS.

摘要

新生儿同种免疫性血小板减少症 (NAIT) 由胎儿与母体血小板不相容引起,导致母体抗体经胎盘转移至胎儿,这些抗体主要针对胎儿人类血小板抗原 (HPA),而由抗人类白细胞抗原 (HLA) 抗体引起的 NAIT 极为罕见。在此,我们报告一例由 HLA 抗体引起的唐氏综合征 (DS) 合并 NAIT。一名 36 周妊娠的 DS 男婴出生,血小板计数降至 13.0×10/L,提示为 NAIT,而非其他情况,包括短暂性髓系造血异常。随机输注血小板浓缩物和静脉注射免疫球蛋白可解决血小板减少症,且无临床并发症。免疫血清学检查发现患者和母亲样本中存在抗-HLA 抗体,但无抗-HPA 抗体。HLA 分型和交叉配型提示,该例患儿的 NAIT 是由先前妊娠期间可能诱导产生的针对父亲 HLA A31 和 B61 的抗-HLA 抗体引起。尽管这种情况较为罕见,但医护人员应考虑 HLA 抗体引起的 NAIT,并警惕后续 DS 病例。

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