1. Approach To Bleeding Disorders In Neonates
هو الشّافی
Approach To Bleeding Disorders In Neonates
S.A.Afjeh (MD)
SBUMS
MMC
1396

2. I) Introduction
Bleeding in well baby is rare BUT in sick baby is serious
Clinical Presentation : ICH, Cephalhematoma, Venipuncture, Bruising, GIB, …
Etiology : Congenital or Acquired
Coagulation Systems :
Cellular (Platelets, …)
Plasma Proteins (Developmental Hemostasis)
Ref : Blood Coagul Fibrinolysis 2011;22:110e7.

3. II) Fetal development of the coagulation system
Ref : Pediatr Clin N Am 2013; 60:1407e17.

4. III) Congenital Hemophilia Type A & B (F VIII & F IX) X Link Recessive
Incidence 1/ /200000, Male
Classification : Severe (< 1%), Moderate (1-5%), Mild(>5-40%)
Circumcision, ICH (Assisted Vaginal Delivery)
Mean Age : 28.5 days, First Month : 70%
F VIII activity in neonates as normal adults but F IX activity is about 50% of adult level at birth
Treatment : Factor Concentration
Ref : Am J Hematol 1998;59:288e94.

5. III) Congenital Von Willebrand Disease (VWD)
Most frequent inherited bleeding disorder
Mucocutaneous & Gum bleeding, Epistaxis, GIB, Bruising
Autosomal Recessive & Dominant, 1% population
Types : 1 (Partial Quantitative Deficiency, Most Common), 2 (Qualitative, Severe Bleeding), 3 (Complete Deficiency, Most Severe Bleeding, Rarest)
Diagnosis
Quantitative VWF in plasma
Quality VWF & ability to bind platelet
Factor VIII activity
VWF is normal or high at birth
RX : pd.F VIII
Ref : Hematology Am Soc Hematol Educ Program 2012;2012:161e7.

6. III) Congenital Rare Inherited Bleeding Disorder Fibrinogen Disorders
Hypofibrinogenemia (Heterozygous) or afibrinogenemia (Aut.R) or dysfibrinogenemia
Prolong umbilical bleeding, post circumcision, ICH, …
Diagnosis : Prolong PT, aPTT, Decreased functional or antigen fibrinogen assay
Treatment : Fibrinogen concentrates, FFP, Cryopercipitate
Ref : Haemophilia 2008;14:1151e8.

7. III) Congenital Rare Inherited Bleeding Disorder
Prothrombin (F II), F V, F VII, F X, F XI Deficiency
Autosomal Recessive
Mucocutaineous Bleeding, Trauma, ICH, Umbilical Bleeding
F VII & XI levels do not correlate with bleeding phenotype
Diagnosis : ↑PT & aPTT (F II, F V, F X), Isolated ↑PT (F VII) & ↑aPTT (F XI), Specific Factor Assay
Treatment :
Factor Concentrate F VII (rF VIIa)
F II, F VII, F IX, F X (Prothrombin Complex Concentrate)
FFP (F V & XI)
F XI in some countries
Ref : J Thromb Haemost 2009;7(Suppl. 1):84e7.

8. III) Congenital Rare Inherited Bleeding Disorder F XIII Deficiency
Autosomal Recessive, Subtype A & B
Umbilical Bleeding (50-70%), Bleeding After Procedure or Trauma
Diagnosis : PT, aPTT, Qualitative F XIII assay, Clot solubility assay (Not sensitive for low level F XIII)
Treatment : pd F XIII, Recombinant F XIII, Cryoprecipitate (If F XIII concentrate is not available)
Ref : J Thromb Haemost 2003;1:1852e3.

9. IV) Acquired Liver Failure
Synthesis of most pro & anti coagulant proteins (Except F VIII & VWF) in liver
Bleeding & Thrombosis
Elevated PT, aPTT, D-dimer
Decreased fibrinogen activity & platelet count
Treatment : FFP, rF VIIa, Cryopercipitate
Ref: Pediatric Blood Cancer 2009;53:179e83.

10. IV) Acquired Vitamin K Deficiency
Platelet dysfunction or thrombocytopenia
DIC
Therapeutic Hypothermia (Total Body or Head Cooling)
Treatment for HIE
Increased Risk of ICH, CBF, ↓ BP, Platelet & Coagulation System Function
Coagulopathy, Thrombocytopenia, Bleeding
Treatment : FFP, Cryopercipitate
Ref: N Engl J Med 2009;361:1349e58.

11. V) Algorithm for the approach to a bleeding neonate
Ref: Seminars in Fetal & Neonatal Medicine 21 (2016) 44e49.

12. VI) Conclusion
Neonates are in a continuous state of developmental hemostasis.
Bleeding in neonates can be challenging to diagnose and treat.
With systematic evaluation of various coagulation tests, the appropriate diagnosis can be made and treatment can be initiated.
Initially, treatment is often supportive, but once a diagnosis is made, it can and should be tailored to specific etiologies.

13. Thanks God