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Approach to the newborn with thrombocytopenia
Dr. Lourdes Asiain Nov 2004
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Objectives Define mild, moderate and severe thrombocytopenia
List most common causes for the condition List dysmorphic features assoc with Thrombocytopenia Description and management of neonatal alloimmune thrombocytopenia
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Thrombocytopenia: Definition
Mild 100,000 to 150,000 Moderate 50,000 to 99,000 Severe Less than 50,000
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Thrombocytopenia: Epidemiology
Up to 25 % of infants admitted to NICU have the condition. Mild asymptomatic thrombocytopenia occurs in 1% healthy term infants Severe thrombocytopenia in term infants rare
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Thrombocytopenia Thrombocytopenia Increased platelet consumption
Impaired Megakaryopoiesis Thrombocytopenia
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Thrombocytopenia: assesment
Term or preterm? Other medical conditions Are there features suggestive of congenital infection? Congenital anomalies/dysmorphism
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Differential Diagnosis
Sick Neonates, Preterm infants, other medical complications. Hypoxia and acidosis after birth Chronic hypoxia from placental insufficiency Hypothermia Pre-eclampsia Sepsis Congenital viral infections (CMV, rubella) DIC NEC Thrombosis Exchange transfusions
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Differential diagnosis
Neonates with physical abnormalities/dysmorphism Thrombocytopenia with absent radius Synd Fanconi anemia Chromosomal disorders (13,18,21, Turner) Kasabach-Merritt Syndrome
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Differential diagnosis
Healthy-appearing infant Occult infection Maternal autoimmune thrombocytopenia Neonatal alloimmun thrombocytopeni Amegakaryocytic thrombocytopenia Hereditary macrothrombocytopenia Wiskott-Aldrich Sydrome
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Common causes Pre-eclampsia
Magnitude usually correlates with severity of HTN Often have associated neutropenia Nadir at 3-4 days of life, recovering to normal levels by day 7-10 Rarely severe thrombocytopenia Possible etiology is disruption of hematopoietic progenitor cell commitment to megakaryopoiesis Assoc conditions from prematurity may exacerbate condition
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Common causes Bacterial Sepsis
Several mechanisms that include endothelial damage, immune mediated destruction, platelet aggregation and decreased platelet production.
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Common causes Viral infections
Viral neuraminidase causes sialic acid loss from platelet membranes Intravascular platelet aggregation Congenital infections + Splenomegaly
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Assoc with Physical abnormalities or dysmorphism
TAR Syndrome Autosomic recessive Severe thrombocytopenia and skeletal abnormalities Can be associated with congenital heart disease Platelet nadir during first week generally improve by 1-3 years of age.
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TAR Syndrome
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Anomalies/Dysmorphism
Fanconi anemia Aplastic anemia Thumb anomalies Anomalies of GU system
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Abnormalities/Dysmorphism
Kasabach-Merrit Syndrome Hemangioma Coagulation is activated locally and platelets are sequestered in the vascular malformation May not be apparent
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Maternal autoimmune Thrombocytopenia
ITP, autoimmune diseases Maternal antibodies vs. platelet antigens Glycoprotein IIb/IIIa and Ib/IX Antibodies cross placenta and bind to these antigens on fetal platelets. Determine the mother’s platelet count
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Neonatal alloimmune thrombocytopenia
A mother lacks a platelet antogen that her fetus inherited from father. Maternal IgG vs “foreign” antigen on fetal platelets cross the placenta and destroy them. Most common platelet antigen HPA-1a
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Management of immune mediated Thrombocytopenia
IVIG In cases of severe thrombocytopenia or clinical bleeding 1gram/Kg In cases of NAIT Head US and platelet transfusion with platelets that are free on HPA1 antigen.
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Less common etiologies
Congenital amegakarocitic thrombocytopenia (AR) Heredetary macrothrmbocytopenias (AD) Wiskott Aldrich Syndrome (WAS protein X) Immunedefiency Eczema Thrombocytopenia
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Conclusions Thrombocytopenia is often encountered in newborns, specially sick preterm infants Initial assessment should consider gestational age, co-morbid conditions, congenital-physical anomalies and dysmorphism.
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Thank You References NeoReviews.org: Approach to the Newborn who has thrombocytopenia. Vol Curr opinion Obst Gynecol: Platelet disorders in pregnancy. 2001;13: Avery’s Diseases of the Newborn. Taesch et Ballard.
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