Neonatal alloimmune thrombocytopenia (NAITP), defined as
thrombocytopenia (platelet count < 150 x 10(9)/l) due to transplacentally acquired maternal platelet
alloantibodies, occurs in approximately 1 per 1200 live births in a Caucasian population. In such a population, the majority (> 75 percent) of cases are due to fetomaternal incompatibility for the platelet specific
alloantigen,
HPA-1a (P1A1, Zwa). Incompatibility for the HPA-5b (Bra)
alloantigen is the next most frequent cause of NAITP in Caucasians; much less common is NAITP due to incompatibility for HLA,
blood group ABO or other
platelet-specific antigens. In non-Caucasian populations (e.g. Orientals)
HPA-1a incompatibility is a rare cause of NAITP and other
alloantigens e.g. HPA-4b (Penb, Yuka) are implicated. The greatest clinical challenge relates to the antenatal management of pregnant women alloimmunized to the
HPA-1a (P1A1, Zwa)
antigen, and particularly the subset of such women who have a history of a previously affected infant with severe
thrombocytopenia and/or
intracranial hemorrhage (ICH). The risk of antenatal ICH in the fetus of such women is high enough to merit intervention, either weekly infusion of high-dose
intravenous immunoglobulin G (
IVIG) with or without
corticosteroids given to the mother (the preferred approach in North American centres), or repeated in-utero fetal
platelet transfusions (the preferred treatment approach in some European centres). Post-natal management of severely affected infants centres on the rapid provision of compatible
antigen-negative platelets harvested from the mother or a phenotyped donor. The value of antenatal screening programs to detect 'at risk' alloimmunized women during pregnancy continues to be debated.