Primary and Secondary Polycythemia
Kristi S. Lively, DVM, DABVP
BASIC INFORMATION
Description
Polycythemia is an elevated red blood cell (RBC) count that is
accompanied by an increased packed cell volume (PCV) and
increased hemoglobin level. Polycythemia may be relative or
absolute. In relative polycythemia, the RBC count is not truly
increased, but the PCV is elevated because there is less fluid
(plasma) in the blood, which makes the relative amount of RBCs
appear to be high. Absolute polycythemia occurs when more RBCs
are produced than normal and their count is truly elevated.
Causes
Dehydration is a common cause of relative polycythemia.
Absolute polycythemia may be primary or secondary. Primary
polycythemia (polycythemia vera) is a spontaneous proliferation
of RBCs in the bone marrow. Secondary polycythemia is induced
by physiologic changes that increase the body’s demand for oxy-
gen, such as certain types of heart disease, living at high altitudes,
lung disease, severe obesity, and defective oxygen transport.
Secondary polycythemia may also be caused by increased
levels of erythropoietin, a hormone that stimulates RBC produc-
tion. Erythropoietin levels may be increased with certain kidney
diseases, and erythropoietin-like substances may be secreted by
certain tumors.
Clinical Signs
With relative polycythemia, vomiting or diarrhea may be pres-
ent and may result in dehydration. Absolute polycythemia usually
occurs in older animals. Signs may include gums that are bright
red, increased drinking and urination, seizures or other behavioral
changes, weakness, hemorrhages within the eyes, retinal detach-
ments, and blindness.
Diagnostic Tests
Once a high RBC count or high PCV is discovered, several addi-
tional tests are usually needed to look for evidence of dehydration
and to determine whether the polycythemia is relative or abso-
lute. Depending on the initial results, tests may be recommended
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to determine the cause of the dehydration, to differentiate primary
from secondary polycythemia, and to look for underlying diseases.
Erythropoietin levels may be checked, but in as many as half of
patients with secondary polycythemia abnormally high levels are
not detected.
X-rays and an abdominal ultrasound are often useful in search-
ing for underlying conditions, such as lung disease, heart disease,
and kidney problems. A bone marrow aspiration or biopsy may be
recommended in cases of primary polycythemia.
TREATMENT AND FOLLOW-UP
Treatment Options
The goals of treatment are to correct any underlying dehydration
and/or causes of secondary polycythemia and to remove contribut-
ing factors whenever possible. Phlebotomy and fluid therapy may
be used in cases of absolute polycythemia to temporarily lower the
PCV, thin the blood, and alleviate clinical signs. Phlebotomy is the
removal of RBCs by drawing blood from a vein (similar to when
a blood sample is taken for laboratory tests but in larger volumes).
Phlebotomy may not be appropriate for patients with diseases that
cause low oxygen levels.
A chemotherapeutic drug, hydroxyurea, may be recommended
for primary polycythemia. Appropriate treatments for any heart and
lung diseases are started. If possible, tumors or masses that may be
secreting erythropoietin-like substances are surgically removed.
Follow-up Care
Periodic phlebotomy maybe required in patients with absolute
polycythemia. The PCV is usually checked weekly until it is sta-
ble, and then periodically. Underlying diseases also require ongo-
ing monitoring.
Prognosis
Prognosis depends largely on the severity of the underlying dis-
ease and response to treatment. Patients with polycythemia are more
prone to complications but may survive for long periods of time
(greater than 1 year).