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Erythrocytosis
Definition: An increase in the
number of circulating RBCs per
volume of blood.
Synonym = polycythemia.
Reflected as an elevated
hemoglobin and hematocrit.
Causes are classified as:
Secondary (Polyclonal)
Primary (clonal)
Secondary Causes of
Increased Red Cell Mass
(Erythrocytosis)
Diagnosis:
Less Common
Bruising/epistaxis
Budd-Chiari syndrome
Erythromelalgia
Gout
Hemorrhagic events
Hepatomegaly
Ischemic digits
Thrombotic events
Transient neurologic
complaints (headache,
tinnitus, dizziness,
blurred vision,
paresthesias)
Atypical chest pain
Diagnosis
PV should be suspected when
hemoglobin and/or hematocrit
levels are elevated
P vera - symptoms
Sx common to all erythrocytosis
Hepatomegaly
40% of pts
Hgb/Hct
WBC in 45%
Plts in 65%
Basophilia (seen in all MPDs)
Labs
Bone marrow studies are not
necessary to establish the
diagnosis but the findings of:
hypercellularity
hyperplasia of the erythroid,
granulocytic and megakaryocytic cell
lines
myelofibrosis
Komplikasi
Examples of thrombotic events
include arterial and venous
thrombosis, cerebrovascular
accident, deep venous thrombosis,
myocardial infarction, peripheral
arterial occlusion, and pulmonary
infarct
P vera - Treatment
Phlebotomy
Myelosuppressive agents
Hydroxyurea
Alkylating agents such as busulfan
32P
Interferon alpha
P vera - phlebotomy
Generally, the best initial treatment
for P vera
No increase in progression to AML
Rapid onset
No BM suppression
P vera - Myelosuppression
Hydroxyurea
32
Benefits
No
No
No
OK
myelosuppression
increase in progression to AML
increase in thrombosis risk
in pregnancy
Drawbacks
Treatment
Occasionally, chemotherapy may
be given to suppress the bone
marrow.
The use of anti-platelet therapy
(such as aspirin) is controversial
because it may cause gastric
bleeding.
Allopurinol is given for
hyperuricemia (gout).
Age > 65 or
h/o
thrombosis
Cardiovascular risk
factors ?
(high cholesterol, DM,
smoking, HTN, CHF)
Recommended treatment
Low
No
No
Intermediate
No
Yes
High
Yes
Treatment
splenectomy in patients with
painful splenomegaly or repeated
episodes of thrombosis causing
splenic infarction
Prognosis
The major causes of morbidity
and mortality are as follows:
Thrombosis
Hemorrhagic complications
Peptic ulcer disease
Myelofibrosis and pancytopenia
Acute leukemia or a myelodysplastic
syndrome