Neonatal Polycythemia

NICU – December 2018

Allison Becker & Induja Gajendran(PGY-2)
 The Patient
 Baby Girl born via NSVD at 39+1 weeks of gestation
 Parity 3003
 Maternal history of Hypothyroidism on Levothyroxine 150mcg daily
 Maternal Labs: RPR negative, Hep B negative, HIV negative, GBS Positive
 Antibiotics: None
 Amniotic Fluid Clear, ROM 8 minutes
 Apgars 9, 9
 Physical exam unremarkable
 The Case
 Admitted to nursery
 A+/A-/Coombs negative
 Routine screening CBC sent from nursery due to GBS positive with no
antibiotics
 Capillary sample: 20.4>24.4/72<256 N54 B4 L21 M10
 Repeat Venous Sample: 19>24.1/73<258 N68 B3 L24 M4
 NICU Course
 Admitted to NICU for management of Polycythemia.
 UV Line Placed
 Partial Exchange Transfusion Completed
 CBC s/p Partial Exchange: 17<19.5/58>264 N63 B6 L13 M8
 Serum bilirubin 4.9@18 hours of life and 7.6@42 hours of life (Low Risk)
 No Hypoglycemia
 Patient discharged home with PMD follow up
Polycythemia
 Definitions
  Polycythemia is an increased total RBC mass. Polycythemic hyperviscosity is an
increased viscosity of the blood resulting from, or associated with, increased numbers
of RBCs.
 Polycythemia of the newborn. Defined as a central venous hct >65%. There is a
curvilinear relationship between the circulating RBC volume (hct) and whole-blood
viscosity. Above a hct of 65%, blood viscosity, rises exponentially.
 Hyperviscosity. Defined as a viscosity >14 cP at a shear rate of 11.5/s measured by a
viscometer. Clinically useful methods of measuring viscosity are currently not available.
Therefore, Hct is used as a proxy for viscosity. Because viscosity is affected by
other factors (e.g., flow rate in small vessels, plasma protein concentrations, acidosis,
red blood cell deformability), viscosity does not correlate directly with Hct.
 Normal newborn physiology 
Compared with older infants and children, term newborns have an
increased red cell mass caused by,

• The fetal response of increased hemoglobin production to a relatively

hypoxic intrauterine environment

• Possible vasomotor instability and venous pooling in newborn

immediately after birth.

• The mean hematocrit (hct) and hemoglobin concentrations

from capillary samples in healthy term infants at birth are 61±7
standard deviations (SD) percent and 19.3±2.2 (SD) g/dL, respectively
 Incidence

  Polycythemia occurs in 2–4% of the general newborn population. Half of

these patients are symptomatic, although it is not at all certain whether
their symptoms are caused by polycythemia.
  Hyperviscosity without polycythemia occurs in 1% of normal
(nonpolycythemic) newborns. In infants with a hematocrit of 60–64%, a
fourth have hyperviscosity
 Pathophysiology

 Blood viscosity depends on the interaction of frictional forces in whole blood,

shear stress and shear rate. The shear rate in the aorta is 230/s and is only
11.5/s in the small arterioles and venules. As the viscosity increases, such as in
the microcirculation, blood with a high hematocrit may virtually cease flowing.
 Regional effects of hyperviscosity: tissue hypoxia, acidosis, and hypoglycemia,
 Formation of microthrombi within the microcirculation.
 Potentially affected organs include the CNS, the kidneys and adrenal glands,
CVS, and the GI
 PATHOPHYSIOLOGY
Hyperviscosity in the newborn contributed by:
 High Hematocrit : from either an absolute increase in circulating RBC volume
or a decrease in plasma volume.
 Plasma viscosity: Plasma proteins, particularly HMW, such as fibrinogen.
Term and, preterm infants have low plasma fibrinogen levels, except for the
rare case of primary hyperfibrinogenemia. Low contribution.
 RBC aggregation: Occurs only in areas of low blood flow and is usually
limited to the venous microcirculation.
 Deformability of RBC membrane: The increase in deformability is presumed
to reduce blood viscosity and to reduce the likelihood that polycythemia will
cause hyperviscosity. Because infants of diabetic mothers are thought to
have RBCs with reduced deformability, hyperviscosity may be more likely at
polycythemic hematocrits than in unaffected neonate.
 Risk factors

1. Conditions that alter incidence :

 Altitude: There is an absolute increase in RBC mass as part of physiologic adaptation to
high altitude.
 Neonatal age:  The normal pattern of fluid shifts during the first 6 hours of life is away from
the intravascular compartment. The period of maximum physiologic increase in the
hematocrit occurs at 2–4 hours of age.
 Obstetric factors:  A delay in cord clamping beyond 30 seconds or stripping of the umbilical
cord, if that is the prevailing practice, results in a higher incidence of polycythemia.
 High-risk delivery: associated with an increased incidence of polycythemia, particularly if
precipitous or uncontrolled.
What causes polycythemia?

The
baby’s
body
makes
more
RBCs than
it should Just after birth,
too many RBCs
traveled from the
umbilical cord to
the baby before it
was clamped
 1. Enhanced fetal erythropoiesis
Placental insufficiency
 Maternal hypertensive disease or
primary renovascular disease
 Abruptio placentae (chronic
recurrent)
 Postmaturity
 Cyanotic congenital heart disease
 Intrauterine growth restriction (IUGR)
 Maternal cigarette smoking
Endocrine disorders. 
 Infant of a diabetic mother (>40%
incidence of polycythemia)
 Infant of a mother with gestational
diabetes (>30% incidence of
polycythemia)
 Congenital thyrotoxicosis
 Congenital adrenal hyperplasia
 Beckwith-Wiedemann syndrome
(secondary hyperinsulinism)
 Genetic trisomies. Trisomies 13, 18, and
21.
 2. Hyper transfusion
 Delay in cord clamping: Placental  Twin–twin transfusion
vessels contain up to 1/3 of the  Maternal-fetal transfusion:
fetal blood volume, half of which
 Approximately 10–80% of normal
is returned to the infant within 1 newborn infants receive a small
minute after birth. volume of maternal blood at the time
 Gravity: Positioning the infant of delivery.
below the placental bed (>10 cm  Intrapartum asphyxia. Prolonged fetal
below the placenta) enhances distress enhances the net umbilical
placental transfusion via the blood flow toward the infant until cord
umbilical vein. clamping occurs, and acidosis may
encourage capillary leak, reduced
 Maternal use of medications.  plasma volume, and decreased red
 Cesarean delivery blood cell deformability.
 Clinical presentation
 Central nervous system: altered state of consciousness: lethargy and decreased
activity, irritability, proximal muscle hypotonia, vasomotor instability, and
vomiting. Seizures, thromboses, and cerebral infarction are extraordinarily rare.
 Cardiopulmonary system: Respiratory distress and tachycardia may be present.
CHF with cardiomegaly and Pulmonary hypertension rare.
 Gastrointestinal tract:  Feeding intolerance. Necrotizing enterocolitis but rarely
without other factors (IUGR).
 Genitourinary tract: Oliguria, acute renal failure/acute kidney injury, renal vein
thrombosis, or priapism may occur.
 Metabolic disorders: Hypoglycemia, hypocalcemia, or hypomagnesemia may be
seen.
 Hematologic disorders: hyperbilirubinemia, thrombocytopenia, or reticulocytosis
(with enhanced erythropoiesis only).
 Skin: Plethora, prolonged capillary filling time
 Diagnosis

 Venous (not capillary) hematocrit: Polycythemia is present when the

central venous hematocrit is ≥65%.
The following screening studies may be used:
 A cord blood hematocrit >56% suggests polycythemia.
 A warmed capillary hematocrit ≥65% suggests polycythemia.
 Management
  Clinical management of the polycythemic infant is more expectant now than a
decade ago. Studies and reviews have created much doubt about any long-term
benefits of partial exchange transfusion (PET). Consequently, PET should probably
be performed only on infants in whom significant morbidity is in question.
 Asymptomatic infants: Only expectant observation is required for virtually all
asymptomatic infants. The possible exception is an infant with a central venous
hematocrit of >75%, but even in this group the risks of central catheter insertion
probably outweigh the benefits of PET.
 Symptomatic infants: When the central venous hematocrit is ≥65%, PET with
normal saline may ameliorate acute signs of polycythemia or hyperviscosity.
 Partial Exchange Transfusion
 Volume=[(Observed Hematocrit-Desired Hematocrit)/Observed Hematocrit]x
Blood vol (ie., Weight in kg x 80)
 Blood is removed and replaced by saline in serial aliquots of 10mL with
removal taking approximately 2.5 minutes and replacement taking
approximately 2.5 minutes for each 10 mL
 Volume exchanged in a term infant is usually 40-60mL
 Easiest way is through a UV Line
 Keep infant NPO for at least 4 hours after procedure
 Repeat CBC at least 4 hours after procedure
 Prognosis

 The long-term outcome of infants with polycythemia or hyperviscosity and

response to PET is as follows:
 A causal relationship exists between PET and an increase in
gastrointestinal tract disorders and necrotizing enterocolitis.
 Older randomized controlled prospective studies of polycythemic and
hyperviscous infants indicate that PET may reduce but not eliminate the
risk of neurologic sequelae. More recent data suggest that no benefits
accrue from PET.
 Infants with “asymptomatic” polycythemia have an increased risk for
neurologic sequelae, but normocythemic controls with the same perinatal
histories have a similarly increased risk.
Copyrights apply
 References
Dempsey EM, Barrington K. Short and long term outcomes following partial exchange transfusion in the polycythaemic newborn: a systematic review.
Arch Dis Child Fetal Neonatal Ed. 2006;91:F2–F6.
Dempsey EM, Barrington K. Crystalloid or colloid for partial exchange transfusion in neonatal polycythemia: a systematic review and meta-analysis. Acta
Paediatr. 2005;94:1650–1655.
Mercer JS, Vohr BR, McGrath MM, Padbury JF, Wallach M, Oh W. Delayed cord clamping in very preterm infants reduces the incidence of intraventricular
hemorrhage and late-onset sepsis: a randomized, controlled trial. Pediatrics. 2006;117:1235–1242.
Morag I, Strauss T, Lubin D, Schushan-Eisen I, Kenet G, Kuint J. Restrictive management of neonatal polycythemia. Am J Perinatol. 2011;28:677–682.
Oh W. Timing of umbilical cord clamping at birth in full-term infants. JAMA. 2007;11:1257– 1258.
Rosenkrantz TS. Polycythemia and hyperviscosity in the newborn. Semin Thromb Hemost. 2003:29:515–527.
Seng YC, Rajadurai VS. Twin-twin transfusion syndrome: a five year review. Arch Dis Child Fetal Neonatal Ed. 2000;83:F168–F170.
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