ABSTRACT
Alloimmune thrombocytopenia (AIT) is characterized by severe thrombocytopenia, usually diagnosed after birth, which may result in intracranial hemorrhage (ICH) in as many as 20% of cases. The course of AIT typically worsens in subsequent pregnancies. Administration to mother of intravenous gammaglobulin (IVIG) and/or corticosteroids to increase the fetal platelet counts of a subsequent affected fetus is widely used to avoid ICH. The objective of this study was to evaluate the long-term effects of AIT and its antenatal treatment on the medical and developmental outcomes of affected children. Seventy-one pairs of untreated (older) and antenatally treated (younger) siblings with AIT were compared. A medical questionnaire and the Behavioral Assessment System for Children (BASC) were completed over the telephone by mothers. In this sample, birth platelet counts of treated fetuses were significantly higher than those of the untreated fetuses. Treated children were born at significantly lower gestational ages and with significantly lower birthweights than untreated children. No treated child suffered a perinatal ICH compared with 12 untreated siblings. Treated siblings also had fewer vision problems (three versus 14 in the untreated group). Children treated as fetuses received higher scores on the BASC Adaptive Skills Composite than their untreated siblings. The antenatal regimen of IVIG and/or corticosteroids did not affect the results. Children with AIT treated as fetuses had better long-term developmental-behavioral outcomes than their untreated siblings, perhaps because of higher in utero platelet counts. We speculate that platelets may possibly play a role in neurodevelopmental processes in the fetus.
Subject(s)
Adrenal Cortex Hormones/adverse effects , Child Development/drug effects , Maternal-Fetal Exchange , Purpura, Thrombocytopenic, Idiopathic/congenital , Purpura, Thrombocytopenic, Idiopathic/drug therapy , gamma-Globulins/adverse effects , Adolescent , Adrenal Cortex Hormones/therapeutic use , Adult , Child , Child Behavior/drug effects , Child, Preschool , Female , Fetal Diseases/prevention & control , Follow-Up Studies , Humans , Infant , Infant, Newborn , Intracranial Hemorrhages/epidemiology , Intracranial Hemorrhages/prevention & control , Platelet Count , Pregnancy , Prenatal Exposure Delayed Effects/epidemiology , Prenatal Exposure Delayed Effects/etiology , Purpura, Thrombocytopenic, Idiopathic/complications , Siblings , Time , gamma-Globulins/administration & dosageABSTRACT
BACKGROUND: Affected patients with neonatal alloimmune thrombocytopenia (AIT) are often severely thrombocytopenic and, if so, may suffer an intracranial hemorrhage (ICH). This study was undertaken to compare the outcome of cases of AIT to cases of neonatal thrombocytopenia shown not to be AIT and to identify clinical features that would facilitate the diagnosis. PROCEDURE: Two hundred twenty two cases of neonatal thrombocytopenia for which serologic testing was obtained by the referring physician were accrued for this study from 11 testing laboratories. The relevant clinical information was pursued. RESULTS: The mean birth platelet count in 110 neonates with AIT was 26,000/mm(3) x 10(9)/L and the rate of ICH was 11% (not all neonates had head sonos). Three criteria distinguished cases of AIT from other causes of neonatal thrombocytopenia (n = 56): (1) severe thrombocytopenia <50,000/mm(3) x 10(9)/L; (2) ICH associated with 1 or more of: a 1-min Apgar score >5, birthweight >2,200 g, grade >1, antenatal occurrence, or signs of bleeding, that is, petechiae, ecchymoses; and (3) no additional, non-hemorrhagic neonatal medical problems. CONCLUSIONS: AIT is a unique type of neonatal thrombocytopenia with significant hemorrhagic consequences. Identification of AIT at the bedside should guide institution of appropriate treatment and lead to serologic testing for confirmation.
