Polycythemia and Hyperviscosity Kirsten E. Crowley, MD June, 2005 Definitions Polycythemia is increased total RBC mass Central venous hematocrit > 65% • Above 65% blood viscosity.
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Transcript Polycythemia and Hyperviscosity Kirsten E. Crowley, MD June, 2005 Definitions Polycythemia is increased total RBC mass Central venous hematocrit > 65% • Above 65% blood viscosity.
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
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
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