[Fetal-neonatal alloimmune thrombocytopenia].
Fetal-neonatal alloimmune thrombocytopenia is the commonest cause of severe thrombocytopenia in the newborn. This disorder is due to the destruction of fetal platelets by a maternal platelet-specific antibody caused by fetal-maternal incompatibility. The most serious complication is intracranial hemorrhage (10-30 % of newborns), which may cause death (10 % of the reported cases) or irreversible neurological sequelae (20 %). The diagnosis is usually made after birth when most affected neonates have petechiae, purpura or overt bleeding. The degree of severity varies according to platelet count. Current methods allow detection of maternal platelet alloantibodies (usually HPA-1a). Clinical grounds and the exclusion of other causes of neonatal thrombocytopenia are required to establish an accurate diagnosis. Recurrence of this disease is very high and has prompted clinicians to develop antenatal prophylactic programs in subsequent pregnancies. However, the optimal treatment of at-risk pregnancies remains controversial. The early diagnosis of this process allows effective therapy based on the infusion of compatible platelets and IgG immunoglobulins when hemorrhage is not obvious. Antenatal management of subsequent pregnancies can prevent recurrence of thrombocytopenia and intracranial hemorrhage. The aim of this review is to draw pediatricians' attention to the importance of this probably under-diagnosed disease in which early diagnosis can prevent potentially severe complications.
Muñiz-Díaz
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Banco de Sangre. Departamento de Hematología. Hospital Sant Pau-Creu Roja. Universidad Autónoma de Barcelona. España. emuniz@hsp.santpau.es
Ginovart Galiana
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G
D016824:Antigens, Human Platelet / Q000145:classification / Q000276:immunology; D003937:Diagnosis, Differential; D006684:HLA-DR Antigens / Q000276:immunology; D059826:HLA-DRB3 Chains; D006801:Humans; D007074:Immunoglobulin G / Q000008:administration & dosage*; D016756:Immunoglobulins, Intravenous; D007231:Infant, Newborn; D039661:Integrin beta3; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology
D016824:Antigens, Human Platelet; D006684:HLA-DR Antigens; D059826:HLA-DRB3 Chains; C061248:ITGB3 protein, human; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D039661:Integrin beta3