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Foetal and neonatal alloimmune thrombocytopaenia

机译:胎儿和新生儿同种免疫血小板减少症

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摘要

Foetaleonatal alloimmune thrombocytopaenia (NAIT) results from maternal alloimmunisation against foetal platelet antigens inherited from the father and different from those present in the mother, and usually presents as a severe isolated thrombocytopaenia in otherwise healthy newborns. The incidence has been estimated at 1/800 to 1/1 000 live births. NAIT has been considered to be the platelet counterpart of Rh Haemolytic Disease of the Newborn (RHD). Unlike RHD, NAIT can occur during a first pregnancy. The spectrum of the disease may range from sub-clinical moderate thrombocytopaenia to life-threatening bleeding in the neonatal period. Mildly affected infants may be asymptomatic. In those with severe thrombocytopaenia, the most common presentations are petechiae, purpura or cephalohaematoma at birth, associated with major risk of intracranial haemorrhage (up to 20% of reported cases), which leads to death or neurological sequelae. Alloimmune thrombocytopaenia is more often unexpected and is usually diagnosed after birth. Once suspected, the diagnosis is confirmed by demonstration of maternal antiplatelet alloantibodies directed against a paternal antigen inherited by the foetuseonate. Post-natal management involves transfusion of platelets devoid of this antigen, and should not be delayed by biological confirmation of the diagnosis (once the diagnosis is suspected), especially in case of severe thrombocytopaenia. Prompt diagnosis and treatment are essential to reduce the chances of death and disability due to haemorrhage. Due to the high rate of recurrence and increased severity of the foetal thrombocytopaenia in successive pregnancies, antenatal therapy should be offered. However, management of high-risk pregnancies is still a matter of discussion.
机译:胎儿/新生儿同种免疫血小板减少症(NAIT)是由母亲对父亲遗传的胎儿血小板抗原进行同种异体免疫而产生的,与母亲体内存在的血小板血小板抗原不同,通常在其他健康的新生儿中表现为严重的孤立性血小板减少症。发病率估计为活产的1/800至1/1 000。 NAIT被认为是新生儿Rh溶血病(RHD)的血小板对应物。与RHD不同,NAIT可以在初次怀孕期间发生。该疾病的范围可能从亚临床中度血小板减少症到新生儿期威胁生命的出血。轻度受影响的婴儿可能没有症状。在患有严重血小板减少症的患者中,最常见的表现是出生时出现瘀斑,紫癜或头状血肿,伴有颅内出血的重大风险(占报道病例的20%),从而导致死亡或神经系统后遗症。同种免疫血小板减少症通常是出乎意料的,通常在出生后被诊断出来。一旦被怀疑,就可以通过证明母体抗血小板同种异体抗体来证实该诊断,该母体抗由胎儿/新生儿遗传的父体抗原。产后管理包括输注不含该抗原的血小板,并且不应通过生物学确认诊断(一旦怀疑可诊断)而延迟,尤其是在严重血小板减少症的情况下。及时诊断和治疗对于减少因出血造成的死亡和残疾机会至关重要。由于连续妊娠中胎儿血小板减少症的高复发率和严重性增加,应提供产前治疗。但是,高风险妊娠的管理仍是一个讨论的问题。

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