Silva Francisco, Morais Sofia, Sevivas Teresa, Veiga Ricardo, Salvado Ramon, Taborda Adelaide
Paediatric Department, Hospital Dr Nélio Mendonça, Funchal, Portugal.
BMJ Case Rep. 2011 Sep 4;2011:bcr0720114563. doi: 10.1136/bcr.07.2011.4563.
Neonatal alloimmune thrombocytopenia is a rare (1/1000-5000 births) life-threatening disorder, caused by fetomaternal incompatibility for a fetal human platelet alloantigen inherited from the father, with production of maternal alloantibodies against fetal platelets, leading to severe thrombocytopenia and potential bleeding. Intracranial haemorrhage is the most feared complication. This report presents the case of a term newborn infant, born from caesarean section after a normal pregnancy, presenting signs of skin bleeding with different ages. Obstetric history included a previous spontaneous abortion after amniocentesis. Severe thrombocytopenia (4×10(9)/l platelets) was found and brain ultrasound showed multiple intracranial haemorrhages. Human platelet antigen (HPA) phenotyping showed maternal negative HPA-1a and paternal positive HPA-1a platelets. Strongly positive anti-HPA-1a and weakly positive anti-human leukocyte antigen class I alloantibodies were found in the mother. Multiple platelet transfusions, intravenous immunoglobulin and corticosteroid were given but favourable response was accomplished only after a compatible platelet transfusion. Brain MRI showed multiple subacute and chronic haemorrhages.
新生儿同种免疫性血小板减少症是一种罕见的(1/1000 - 5000活产儿)危及生命的疾病,由胎儿从父亲遗传而来的人类血小板同种抗原引起的母胎不相容性导致,母体产生针对胎儿血小板的同种抗体,进而导致严重血小板减少和潜在出血。颅内出血是最可怕的并发症。本报告介绍了一例足月新生儿的病例,该婴儿在正常妊娠后经剖宫产出生,在不同年龄段出现皮肤出血迹象。产科病史包括之前羊膜穿刺术后的自然流产。发现严重血小板减少(血小板计数4×10⁹/L),脑部超声显示多处颅内出血。人类血小板抗原(HPA)表型分析显示母体HPA - 1a阴性,父亲HPA - 1a阳性血小板。在母亲体内发现抗HPA - 1a强阳性和抗人类白细胞抗原I类同种抗体弱阳性。给予多次血小板输注、静脉注射免疫球蛋白和皮质类固醇,但仅在输注相容血小板后才取得良好反应。脑部MRI显示多处亚急性和慢性出血。