Objective: Fetal and neonatal alloimmune thrombocytopenia (AIT) caused by f
ete-maternal incompatibility at the HPA-la (PLA-I) locus is well characteri
zed. Alloimmunization and disease caused by HPA-3a is rare.
Study design: We conducted a retrospective analysis of all known cases of A
IT caused by HPA-3a incompatibility identified at 3 major reference laborat
ories from 1986 to 1996. Platelet antigen typing and antibody specificity w
ere determined by serologic evaluation. In some cases confirmatory genotypi
ng was performed.
Results: Fourteen cases of anti-HPA-Sa-induced AIT in 11 families were iden
tified. Five patients had a previous affected sibling, and 2 cases were fir
stborn children. All patients had severe thrombocytopenia at birth (platele
t count <20 x 10(9)/L). Regardless of therapy, the median time to platelet
recovery was 6 days (range, 3 to 23 days). Two (15%) patients had documente
d intracranial hemorrhage, 1 with severe sequelae including apnea and convu
lsions. A literature review describing 16 additional patients corroborates
the finding of severe thrombocytopenia and a significant incidence of intra
cranial hemorrhage caused by HPA-3a incompatibility.
Conclusion: AIT caused by incompatibility of HPA-3a is similar in severity
to disease caused by incompatibility of HPA-la. Affected families should be
appropriately counseled and considered for antenatal therapy.