Neonatalogy blood disorder

1. COLLEGE OF HEALTH SCIENCE
DEPARTMENT OF MIDWIFERY
FOR POSTABASIC
COURSE NEWBORNCARE

2. Objectives
At end of this topic student able to :-
Know what is Haematological disorders in the fetus and newborn
Common Haematological disorders occur in newborn?
Identify pathological cause of anemia
Understand clinical manifestation, diagnosis and management of
anemia
Understand the difference between anemia and polycythemia
Describe the bleeding Acquired Bleeding Disorders

3. Hematological disorders
Haematological disorders in the fetus and newborn blood infant
arise from the conditions which primarily constituents of blood
such as red blood cells (RBCs), haemoglobin and platelets.
 Some of the conditions are inherited and amenable to prenatal
diagnosis or screening at birth.
Symptoms in affected infants may start either in utero or after
birth.
Diagnosis of some of the haematological conditions may be
complex, requiring interpretation by a paediatric haematologist.
Treatment includes prevention and specific therapies.

4. Anaemia
 Central venous hemoglobin level < 13 g/dL in an infant of > 34 weeks gestation is
considered anemia.
 Anaemia is pathologic when the tissues do not receive enough oxygen delivery to meet
demands
 At birth normal values of hemoglobin (central venous) in infants > 34 weeks gestation are
14–20 g/dL with an average value of 17 g/dL
 Three basic pathophysiologic causes
 Haemorrhage (internal/external – including excessive phlebotomy)
 Increased destruction (hemolysis)
 Decreased production
 Time of cord clamping (early vs late) & position of infant relative to placenta have major
effects on circulating blood volume & hemoglobin concentration
 Normally infants receive half placental blood volume (30–50 mL) in 1 min
 Infants held above placenta (e.g., during cesarean section) lose 20–30 mL/min back into
placenta

5. Anaemia
Anaemia is usually defined by a haemoglobin (or haematocrit) level
 mild if the haemoglobin level (Hb) is 10– 12,
 moderate if it is between 8–10, and
 severe if it is less than 8 g dl–1 .
The causes of neonatal anaemia
 Physiological
 Haemorrhage
 Haemolysis
 Hypoplasia or aplasia (diminished production)
NB Physiologic anemia of infancy is due to shorter life span of RBCs and
less erythropoietin production

6. 1.Anemia due to blood loss
is characterized by a normal bilirubin level (unless the
hemorrhage is retained).
If blood loss is recent (e.g., at delivery), the hematocrit (Hct)
and reticulocyte count may be normal, and the infant may be
in shock. The Hct will fall later because of hemodilution.
If the bleeding is chronic, the Hct will be low, the reticulocyte
count up, and the baby normovolemic.

7. 1.Anemia due to blood loss…
Obstetric causes of blood loss
a. Incision of the placenta at the time of cesarean section
b. Rupture of anomalous vessels (e.g., vasa previa, velamentous
insertion of the cord, or rupture of communicating vessels in a
multilobed placenta)
c. Hematoma of the cord caused by varices or aneurysm
d. Rupture of the cord (more common in short cords and in
dysmature cords)
e. Abruptio placentae
f. Placenta previa (uncommon)

8. 1.Anemia due to blood loss..
Occult blood loss
 Fetomaternal bleeding diagnosed Kleihauer–Betke stain of
maternal smear for fetal cells
 Obstetric procedures—traumatic amniocentesis, external cephalic version,
internal cephalic
 Fetoplacental bleeding
 Chorioangioma or choriocarcinoma with placental hematoma
 Cesarean section, with infant held above the placenta
 Twin-to-twin transfusion

9. 1.Anemia due to blood loss..
Bleeding in the neonatal period
Intracranial bleeding
Massive cephalohematoma, subgaleal hemorrhage, or
hemorrhagic caput succedaneum
Ruptured liver or spleen ,renal hemorrhage, Gastrointestinal
bleeding
Bleeding from the umbilicus

10. 2.Hemolysis
Hemolysis is manifested by a decreased Hct, an increased reticulocyte
count, and an increased bilirubin level.
 Immune hemolysis
– Rh incompatibility
– ABO incompatibility
– Minor blood group incompatibility (e.g., c, E, Kell, Duffy)
– Maternal disease (e.g., lupus), autoimmune hemolytic disease
Hereditary RBC disorders - RBC membrane defects such as
spherocytosis, elliptocytosis, or stomatocytosis
Acquired hemolysis
 Infection—bacterial or viral
Disseminated intravascular coagulation

11. 3.Diminished RBC production
Diminished RBC production is manifested by a decreased Hct,
decreased reticulocyte count, and normal bilirubin level.
– Physiologic anemia or anemia of prematurity
– Congenital leukemia (very rare)
– Infections, especially rubella and parvovirus
– Osteopetrosis, leading to inadequate erythropoiesis, presents in
infancy.

12. Risk factors
Prematurity
 certain race and ethnic groups,
hereditary blood disorders

13. Clinical presentatio
 Pallor
 Jaundice from hemolysis
 Apnea and bradycardia
 Tachycardia
 Heart murmur
– systolic flow murmur
 Respiratory distress
 heart failure
 Hepatomegaly and/or splenomegaly,
 hydrops
 Inadequate weight gain from poor feeding

14. DIAGNOSTIC APPROACH TO ANEMIA IN THE NEWBORN
The family history should include questions about anemia,
jaundice, gallstones, and splenectomy
The obstetric history should be evaluated for severe abdominal
pain (abruptio) or intrapartum blood loss
The physical examination .
pallor, respiratory distress or irritability
 pallor, jaundice, and hepatosplenomegaly
Capillary blood Hct is 3.7% to 2.7% higher than venous Hct.
 Complete blood cell count

15. DIAGNOSTIC APPROACH TO ANEMIA IN THE NEWBORN
Reticulocyte count (elevated with chronic blood loss and
hemolysis, depressed with infection and production defect)
Peripheral blood smear
Coomb’s test and bilirubin level
Kleihauer–Betke maternal circulation will show up as 1%
fetal cells in the maternal circulation.
Ultrasound of the abdomen and head

16. Causes and investigation of anemia

17. Causes and investigation of anemia

18. Management
Tx of neonatal anemia may involve individually or in
combination,
simple replacement transfusion,
exchange transfusion,
nutritional supplementation, or
 treatment of the underlying primary disorder.

19. Management…
 Simple replacement transfusion
Indications
Acute hemorrhagic anemia.
Ongoing deficit replacement.
Maintenance of effective oxygen-carrying capacity.
There are no universally accepted guidelines; however, those
presented next are fairly representative of most common
practice

20. Management…
Exchange transfusion: blood trasnfusion in which the patient
blood or component of it are exchanged with other blood /
blood product-Indications
 Chronic hemolytic anemia or hemorrhagic anemia with evidence of
tissue hypoxia (poor perfusion, metabolic acidosis, oliguria).
Severe isoimmune hemolytic anemia

21. Management…
Nutritional replacement
Iron replacement (Oral iron in suspension (2–4 mg elemental iron/kg) is
useful in the following situations:
 Fetomaternal hemorrhage of significant volume.
 Chronic twin-twin transfusion (in the donor twin).
 Incremental external blood loss (if unreplaced).
 Preterm infant (<36weeks of GA)
Folate. folic acid 50 mcg/day) ---Especially with serum
levels(<0.5ng/ml)
 Premature infants weighing <1500kg or 34wks GA
 Chronic hemolytic anemias

22. Management…
Treatment of selected disorders
Treat the underlying cause (eg, sepsis).
Give blood replacement therapy.
 Perform exchange transfusion or give fresh-frozen plasma, 10
mL/kg every 12 to 14 hours.
 Platelet concentrate, 1 U, may be used as a substitute for plasma
transfusion.

23. Management…
Prophylaxis
– Delay cord clamping
– Iron supplementation in the preterm infant to improves iron stores,
and lowers the risk of iron deficiency anemia
– Erythropoiesis-stimulating agents

24. Polycythemia
 Is defined as a venous haematocrit of 65% or more, approximating to a
haemoglobin of 22 g/dl (220 g/l ), during the first week of life.
Blood viscosity depends largely on the packed cell volume (haematocrit),
but the deformability of RBCs and the plasma viscosity may also be
significant factors.
The relationship between viscosity and haematocrit is linear below a
haematocrit of 60–65%, but increases exponentially above this level.
 occur in 1-5% of neonate
Polycythaemia should only be diagnosed on a free-flowing venous
specimen and not from a heel-prick sample

25. Causes of neonatal polycythaemia
 Increased circulating RBC secondary to increased production
 Increased fetal erythropoiesis
o Chronic intrauterine hypoxia:
• SGA infants
• Postmaturity
 Excessive transfusion of blood: increased blood volume
– Placental transfusion due to delayed clamping
– Twin-to-twin transfusion
– Maternofetal transfusion
 Infants of diabetic mothers
 Down’s syndrome
 Neonatal thyrotoxicosis
 Congenital adrenal hyperplasia.

26. CLINICAL FINDINGS
Plethoric infant

27. CLINICAL FINDINGS…
Most infants with polycythemia are asymptomatic.
 Central nervous system (CNS).
– Poor feeding, lethargy, hypotonia, apnea, tremors, jitteriness,
seizures, and cerebral venous thrombosis
Cardiorespiratory.- Cyanosis, tachypnea, heart murmur,
congestive heart failure, cardiomegaly, elevated pulmonary
vascular resistance, and prominent vascular markings on chest
x-ray

28. CLINICAL FINDINGS…
Renal - Decreased glomerular filtration, decreased sodium
excretion, renal vein thrombosis, hematuria, and proteinuria
Other -thrombosis, thrombocytopenia, poor feeding,
increased jaundice, persistent hypoglycemia, hypocalcemia,
necrotizing enterocolitis (NEC

29. DIAGNOSIS
Peripheral venous hematocrit sample is preferred to measure
polycythemia.
 Arterial blood sample is not acceptable for hematocrit
estimation as it would underestimate the hematocrit;
Capillary sample would overestimate the hematocrit by 5% to
15%.
However, capillary hematocrit can be used for initial
screening, which should always be confirmed with a venous
hematocrit if greater than 65%

30. Treatment
Treatment is to reduce the hematocrit by replacing
a proportion of the infant’s blood with 0.9% saline (plasma is
no longer used to minimize blood product usage), by partial
exchange transfusion
 venous hematocrit > 0.65 and infant symptomatic or
hematocrit > 0.70 even if asymptomatic – generally agreed
that a partial dilutional exchange transfusion should be
performed.
 If venous hematocrit 0.65–0.70 and asymptomatic – observe
and treat only if becomes symptomatic

31. Treatment..
Fluid management. In neonates with hematocrit >65% and
mild symptoms and with evidence of dehydration, increasing
the daily maintenance fluids by 10 to 20 mL/kg and re-
evaluating after 4 to 6 hours might be a reasonable alternative
option.

32. Acquired Bleeding Disorders: Vitamin K Deficiency Bleeding
Is a fat-soluble vitamin
Is required for carboxylation of glutamic acid residues on
precursors of vitamin K–dependent coagulation proteins (factors
II, VII, IX, and X and proteins C and S).
Vitamin K exists in 2 forms: vitamin K1 or phylloquinone (the
plant form of the vitamin) and vitamin K2, a series of compounds
synthesized by bacteria and referred to as menaquinones.
In contrast to human milk, infant formula contains large amounts
of vitamin K (10 vs 65–100 mcg/L).

33. Acquired Bleeding Disorders: Vitamin K Deficiency Bleeding…
Newborn infants are at risk for vitamin K deficiency because
of;-
– vitamin K’s poor placental transfer
– insufficient endogenous production from the intestinal bacterial
flora prior to complete colonization of the neonatal colon
– inadequate dietary intake among solely breast-fed infants.

34. Risk factors
– liver disease
– cholestasis
– maternal short-gut syndrome
Clinical presentation
• mild - Manifests as an isolated prolongation of PT,
• more severe deficiency characterized by prolongation of
aPTT.

35. diagnosis
Can be confirmed by high serum level of an abnormal form of
prothrombin (protein induced by vitamin K absence

36. Management
Infants who present with a non–life-threatening bleed only need to
be treated with vitamin K1 given slowly intravenously (IV) or
subcutaneously (no IM injection) at a dose of 250 to 300 mcg/kg
to restore PT to 30% to 50% of its normal value within an hour.
Treatment of serious bleeding includes FFP (20 mL/kg), a
prothrombin complex concentrate (50 U/kg), or recombinant
factor VIIa (100 mcg/kg).
 The American Academy of Pediatrics recommends that all infants
receive 1 mg of IM vitamin K on the first day of life (0.3 mg for
infants 1000 g

37. Reading assignment
• Thrombocytopenia
• Thrombophilia

38. • Which of the following is a known complication of delayed
cord clamping? a. Plethora b. Petechiae c. Neonatal anemia
d. Hyperbilirubinemia
• What is the American College of Obstetricians and
Gynecologists’ recommended dose of vitamin K for routine
prophylaxis against hemorrhagic disease of the newborn?
• Which of the following is not a vitamin K–dependent clotting
factors