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) Higher plasma proteins = increased viscosity
Especially fibrinogen (typically low in neonates)

Plasma viscosity

Not usually an issue in neonates

RBC aggregation

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

Hypoglycemia is common with polycythemia

Increased bili due to increased RBC turnover Usually high if baby is deyhdrated

Serum bilirubin

Serum sodium, BUN, urine specific gravity

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