Neonatal alloimmune thrombocytopenic purpura and congenital porencephaly in
two siblings associated with a "new" maternal antiplatelet antibody
JM Friedman and RH Aster
We report a brother and sister, both of whom have porencephaly,
hydrocephalus, optic atrophy, severe mental retardation, and spastic
quadriplegia. In the younger child, abnormal intracranial structure was
demonstrated by sonography at 32 weeks' gestation and was suspected
earlier. Both children had transient severe thrombocytopenia as newborns.
The mother is healthy and has never had purpura or other bleeding symptoms.
However, her serum was found to react strongly with platelets from the
father and from both children. The antibody in the mother's serum is
platelet-specific but does not appear to be directed against any of the
known antigens associated with neonatal alloimmune thrombocytopenic purpura
(NATP) in other families, including PlA1, PlE2, or Baka. Although the
mother's serum reacts with platelets from all 47 unrelated normal donors
tested and from both the mothers and the fathers of 17 other children with
suspected NATP, it does not react with her own platelets or with platelets
from a patient with Glanzmann's thrombasthenia. These observations suggest
that the serum from this woman identifies a previously undescribed
high-frequency platelet-specific alloantigen and that sensitization to this
determinant caused severe immune thrombocytopenia in both her children. It
is likely that this led to intracranial hemorrhage in utero in these cases.
Volume 65,
Issue 6,
pp. 1412-1415,
06/01/1985
Copyright © 1985 by The American Society of Hematology
