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新生儿同种免疫性血小板减少症的一种罕见表现。

A rare manifestation of neonatal alloimmune thrombocytopaenia.

作者信息

Jerónimo Monica, Azenha Cátia, Mesquita Joana, Pereira Dolores Faria

机构信息

Department of Neonatal Intensive Care Unit, Maternidade Bissaya Barreto-Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Department of Ophthalmology, Hospital Pediátrico-Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

出版信息

BMJ Case Rep. 2014 Jun 2;2014:bcr2014204393. doi: 10.1136/bcr-2014-204393.

Abstract

Neonatal alloimmune thrombocytopaenia (NAIT) results from a fetomaternal incompatibility with maternal sensitisation against a fetal human platelet antigen (HPA) and antibodies transfer to the fetal circulation, leading to platelet destruction. The clinical presentation is variable and isolated intraocular haemorrhage is rare. We present the case of a male newborn, with intrauterine growth restriction, born at 29 weeks due to pre-eclampsia. He presented proptosis of the left eye, hyphaema and elevated intraocular pressure, with no other signs of haemorrhage. Severe thrombocytopaenia was found (27×10(9)/L). Perinatal infection and maternal thrombocytopaenia were excluded. Positive anti-HPA-1a and antihuman leucocyte antigen class I alloantibodies were found in the mother. Platelet crossmatch between the father's platelets and mother's plasma was positive. Platelet transfusions and intravenous immunoglobulin were given with favourable response. This case highlights an unusual presentation of NAIT, which should be suspected in the presence of severe thrombocytopaenia in the first 24-72 h of life.

摘要

新生儿同种免疫性血小板减少症(NAIT)是由母胎不相容引起的,母体对胎儿人类血小板抗原(HPA)致敏,抗体转移至胎儿循环,导致血小板破坏。临床表现多样,孤立性眼内出血罕见。我们报告一例男性新生儿病例,该患儿有宫内生长受限,因子痫前期于29周出生。他出现左眼突出、前房积血和眼压升高,无其他出血迹象。发现严重血小板减少(27×10⁹/L)。排除围产期感染和母体血小板减少症。母亲体内发现抗HPA-1a和抗人类白细胞抗原I类同种抗体呈阳性。父亲血小板与母亲血浆的血小板交叉配型呈阳性。给予血小板输注和静脉注射免疫球蛋白,反应良好。该病例突出了NAIT的一种不寻常表现,在出生后最初24 - 72小时内出现严重血小板减少时应怀疑该病。

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