Definition
- Platelet count <150,000 in a neonate
Background
- Fetuses begin making platelets 5 days post conception and reach adult levels by 22w gestation
- For this reason, the definition of thrombocytopenia has the same parameters as adults
- Platelet counts increase with increasing postnatal age
- Recent studies questioning the validity of using adult parameters for neonates, but no change in definition yet
- Normal term neonates have incidence of thrombocytopenia <1% while the incidence for NICU admits is 18-35%
- Most cases are mild and resolve within 7-14 days, but a small subset of cases (2.5-5%) are severe and take months to resolve
- Severe, persistent, and symptomatic patients must be evaluated
Etiology
- Increased Platelet Destruction
- Alloimmune thrombocytopenia: maternal ab against fetal platelets
- Neonatal Alloimmune Thrombocytopenic Purpura (NATP): fetal platelets contain an antigen inherited from the father that the mother lacks. Mother makes IgG against the paternal antigen which crosses the placenta and causes thrombocytopenia
- Infants are at risk for intracranial hemorrhage. 25-50% occur in utero
- Autoimmune thrombocytopenia: maternal ab against maternal and fetal platelets
- SLE, ITP: maternal ab against maternal and fetal platelets
- Drug-related: maternal or fetal ab
- antiepileptics, quinidine, PCN, heparin, hydralazine, thiazides
- Peripheral Consumption
- Hypersplenism
- Kasabach-Merritt Syndrome: DIC with capillary hemangiomas
- DIC
- Infection: bacterial, viral, or fungal.
- Necrotizing enterocolitis
- Thrombosis
- Hemorrhage (most IVH, pulm, or GI)
- premature IVH: normal platelet counts at time of bleed, so causal relationship difficult to determine
- term IVH: thrombocytopenia common at time of bleed
- Alloimmune thrombocytopenia: maternal ab against fetal platelets
- Decreased Platelet Production
- Genetic Disorders
- Thrombocytopenia-absent radius syndrome: AR, bilateral absent radii, thumbs present
- Fanconi’s Anemia
- Congenital amagakaryocytic thrombocytopenia
- Chromosome abnormalities: trisomies (21, 18, 13), Turner syndrome
- Congenital platelet disorders: Wiskott-Aldrich, May-Hegglin, Bernard-Soulier, Alport
- Infiltrative Disorders: neoplasms. Rare in the neonatal period.
- Toxic injury to megakaryocytes by infections/drugs
- Genetic Disorders
- Miscellaneous Causes
- Neonatal Cold injury: mechanism unclear
- Asphyxia: mechanism unclear, but possibly related to hypoxic injury
- Von Willebrand Disease: platelet aggregation
- Preeclampsia: nadir at 2-4 days with resolution typically by 7-10 days
- Dilution
Management
- Initial evaluation
- Thorough history and physical exam of mother and neonate, including birth history
- Healthy appearing infant more likely to have congenital/immunologic mediated cause
- Ill appearing infant more likely to have infection, DIC, NEC, asphyxia as cause
- Labs:
- Neonate: CBC (repeated to confirm thrombocytopenia), platelet ag typing, peripheral smear, coags, sepsis work up if ill appearing, eval for congenital infection, genetic testing
- Maternal: CBC (repeated to confirm thrombocytopenia if present), platelet ag typing
- Cranial US to evaluate for IVH. If present, low threshold for transfusion. Repeat prior to discharge
- Thorough history and physical exam of mother and neonate, including birth history
- Treatment
- If underlying cause known (mom with SLE, ITP, known infection, etc), treat.
- Platelet counts should be more aggressively maintained for first 72-96 hours of life as risk for IVH highest in this time period. If no IVH, then clinical situation used to guide need for transfusion
- High dose IVIG for 3-4 days is used as adjunct
- Methylprednisolone used only in emergency situations as evidence lacking
- When to transfuse?
- No clear guidelines
- Term infant, healthy, no other risks for hemorrhage: typically if <30,000
- Preterm, ill, or with other risk factors: typically if <50,000
- Benefit of platelet transfusion not clearly established; some recent suggestion that transfusion may be harmful
- Other therapies
- IVIG, steroids, exchange transfusion typically used for immune mediated causes
- Current research into using thrombopoeitic mimetics as an alternative to transfusion
References
- Murphy S. Consultation with the Specialist. Thrombocytopenia. Pediatrics in Review. Feb 1999; 20(2): 64-68.
- Albert TS. Throbopoietin in the Thrombocytopenic Term and Preterm Newborn. Pediatrics. June 2000; 105(6): 1286-1291.
- Silver RM. Neonatal Alloimmune Thrombocytopenia: Antenatal Management. American Journal of Obstetrics and Gynecology. May 2000; 182(5): 1233-1238.
- Behrman: Nelson Textbook of Pediatrics, 16th ed (2002). Philadelphia: W.B. Saunders Company.
- Gomella: Neonatology: Management, Procedures, On-Call Problems, Diseases and Drugs, 4th ed (1999). McGraw-Hill/Appleton and Lange.
- Ferrar-Marin F, et al. Neonatal Thrombocytopenia and Magakaryocytopoeisis. Seminar in Hematology. July 2010; 47 (3) 281-288
- UpToDate. Neonatal Thrombocytopenia.