Hinkel G K, Schneider I, Gebhardt B, Leverenz S
Schweiz Med Wochenschr. 1985 Jun 15;115(24):831-6.
Alloimmune neonatal neutropenia is a rare disease. A family with four affected newborns is described in which the course of the disease aggravated from the first to the third child. The third child died from generalized infection. In the mother's serum specific antibodies against neutrophils (anti-NA 1) were detected which reacted with cell suspensions from the father and the other children. In the fourth child no neutrophils were present even immediately after birth. In this child the diaplacental transferred antibodies were detectable in falling concentrations over 8 weeks. The number of neutrophils normalized when the antibodies disappeared. Infection in the newborn was prevented by care in an environment poor in bacteria and by antimicrobial prophylaxis. Repeated exchange transfusions with NA 1-free blood only lowered the antibody titer. Transfusions of white blood cell concentrates had only shortlived effects on the neutropenia.
同种免疫性新生儿中性粒细胞减少症是一种罕见疾病。本文描述了一个有四名患病新生儿的家庭,其中疾病进程从第一个孩子到第三个孩子逐渐加重。第三个孩子死于全身性感染。在母亲血清中检测到针对中性粒细胞的特异性抗体(抗 - NA 1),该抗体与父亲及其他孩子的细胞悬液发生反应。第四个孩子出生后即刻就没有中性粒细胞。在这个孩子中,经胎盘转移的抗体在8周内浓度逐渐下降,仍可检测到。当抗体消失时,中性粒细胞数量恢复正常。通过在细菌含量低的环境中护理和抗菌预防措施,预防了新生儿感染。用不含NA 1的血液反复进行换血仅降低了抗体滴度。输注白细胞浓缩物对中性粒细胞减少症仅产生短暂影响。