Evans D I
J Clin Pathol. 1987 Mar;40(3):258-61. doi: 10.1136/jcp.40.3.258.
Three newborn babies with congenital immune amegakaryocytic thrombocytopenia are described. Two were siblings. The mothers' sera showed antibody against both pooled random platelets and lymphocytes of type HLA-A2. Two babies had transient thrombocytopenia and leucopenia and made a full haematological recovery. One baby died aged four hours having had no haematological investigations during life. Histological examination of the bone marrow in all three babies (two by trephine biopsy; one at necropsy) confirmed a deficiency of megakaryocytes. It is suggested that anti-HLA-A2 may cause neonatal thrombocytopenia by depressing megakaryocytes instead of, or in addition to, any direct effect on platelets.
本文描述了三名患有先天性免疫性血小板减少性紫癜的新生儿。其中两名是兄弟姐妹。母亲的血清显示对混合的随机血小板和HLA - A2型淋巴细胞均有抗体。两名婴儿出现短暂性血小板减少和白细胞减少,并在血液学方面完全恢复。一名婴儿在出生后四小时死亡,生前未进行血液学检查。对所有三名婴儿的骨髓进行组织学检查(两名通过环钻活检;一名通过尸检)证实巨核细胞缺乏。有人提出,抗HLA - A2可能通过抑制巨核细胞而非直接作用于血小板,或两者兼而有之,从而导致新生儿血小板减少。