Polycythemia and
Hyperviscosity
Kirsten E. Crowley, MD
June, 2005
Definitions
 Polycythemia

is increased total RBC mass
Central venous hematocrit > 65%
• Above 65% blood viscosity rises exponentially
 Polycythemic
hyperviscosity is increased
viscosity of the blood resulting from
increased numbers of RBCs

Not all polycythemic infants have symptoms of
hyperviscosity
Incidence
 Polycythemia

occurs in 2-4% of newborns
Half of these are symptomatic
 Hyperviscosity
occurs in 25% of infants
with hematocrit 60-64%

Hyperviscosity without polycythmia occurs in
1% (nonpolycythemic hyperviscosity)
Pathophysiology

Clinical signs result from regional effects of
hyperviscosity and from the formation of
microthrombi
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
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Tissue hypoxia
Acidosis
Hypoglycemia
Organs affected: CNS, kidneys, adrenals,
cardiopulmonary system, GI tract
What affects hyperviscosity?

Hematocrit



Increased hct is the most important single factor
Results from increase in circulating RBCs or
decreased plasma volume (dehydration)
Plasma viscosity

Higher plasma proteins = increased viscosity
• Especially fibrinogen (typically low in neonates)


RBC aggregation



Not usually an issue in neonates
Occurs in areas of low blood flow = venous
microcirculation
Not a large factor in neonates
Deformability of RBC membrane: usually
normal
Conditions that alter
incidence
 Altitude:
increased RBC mass
 Neonatal age

Physiologic increase in hematocrit due to fluid
shifts away from intravascular compartment
with maximum at 2-4 hours of age
 Obstetric
factors: delayed cord clamping
or “stripping” of the umbilical cord
 High-risk delivery, especially if precipitous
Perinatal processes
 Enhanced
fetal erythropoiesis usually
related to fetal hypoxia

Placental insufficiency
• Maternal hypertension, abruption, post-dates,
IUGR, maternal smoking

Endocrine disorders: due to increased oxygen
consumption
• IDM (>40% incidence), congenital thyrotoxicosis,
CAH, Beckwith-Wiedemann syndrome
(hyperinsulinism)
Hypertransfusion

Delayed cord clamping
• Placental vessels contain 1/3 of the fetal blood volume,
half of which will be returned within 1 minute

Gravity: positioning below the placenta will
increase placental transfusion
 Meds: oxytocin can increase contractions and
thus transfusion
• Decreased in c-section b/c no contractions

Twin-twin transfusion
 Maternal-fetal transfusion
 Intrapartum asphyxia
• Enhances net umbilical flow toward the infant, while
acidosis increases capillary leak leading to reduced
plasma volume
Clinical presentation






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Symptoms are non-specific!
CNS: lethargy, hyperirritability, proximal
muscle hypotonia, vasomotor instability,
vomiting, seizures, cerebral infarction (rare)
Cardiopulmonary: respiratory distress,
tachycardia, CHF, pulmonary hypertension
GI: feeding intolerance, sometimes NEC
GU: oliguria, ARF, renal vein thrombosis,
priapism
Metabolic: hypo-glycemia/-calcemia/magnesemia
Heme: hyperbili, thrombocytopenia
Skin: ruddiness
Diagnosis
 Central
venous hematocrit > 65%
 ALWAYS draw a central venous sample if
the capillary hematocrit is > 65%

Warmed capillary hematrocrit > 65% only
suggestive of polycythemia
Management

Asymptomatic infants


Expectant observation unless central venous
hematocrit >75% (consider partial exchange
transfusion)
Can do a trial of rehydration over 6-8 hr if dehydrated
• Usually at > 48 hours of age and weight loss > 8-10%
• Give 130-150 ml/kg/d

Check central hematocrit q6 hours
• Normal peak is at 2-4 hours of age for acute polycythemia
Management

Symptomatic infants with central hct > 65%



Partial exchange transfusion is advisable but
debatable
For exchange can use normal saline, Plasmanate,
5% albumin, or FFP
Volume exchanged =
• (Weight (kg) x blood volume) x (hct - desired hct) / hct


Blood volume is 80 ml/kg
Exchange can be done via UVC that is not in the liver,
low UAC, or PIV
Other labs to check

Serum glucose


Serum bilirubin


Increased bili due to increased RBC turnover
Serum sodium, BUN, urine specific gravity


Hypoglycemia is common with polycythemia
Usually high if baby is deyhdrated
Blood gas to rule-out inadequate oxygenation
as cause of symptoms
 Platelets, as thyrombocytopenia can be
present
 Serum calcium b/c hypocalcemia can be
seen
Prognosis

Increased risk of GI disorders and NEC with
partial exchange transfusion (PET)
 Older trials show decreased neurologic
complications from hyperviscosity with PET, but
newer trials show no real benefit


PET is controversial!
Infants with asymptomatic polycythemia have an
increased risk for neurologic sequelae

Normocythemic controls with the same perinatal
history have a similarly increased risk