Polycythemia vera (pol-e-sigh-THEE-me-uh VEER-uh) is a slow-growing type of

blood cancer in which your bone marrow maes too many red blood cells!
Polycythemia vera may also result in production of too many of the other
types of blood cells " white blood cells and platelets! These e#cess cells
thicen your blood and cause complications$ such as such as a ris of blood
clots or bleeding!
Polycythemia vera isn%t common! &t usually develops slowly$ and you may
have it for years without noticing signs or symptoms! 'ften$ polycythemia
vera is found during a blood test done for some other reason!
(ithout treatment$ polycythemia vera can be life-threatening! However$ with
proper medical care$ many people e#perience few problems related to this
disease! 'ver time$ there%s a ris of progressing to more-serious blood
cancers$ such as myelo)brosis or acute leuemia!
*ymptoms
+y ,ayo -linic *ta.
/or many people$ polycythemia vera may not cause any signs or symptoms!
However$ some people may e#perience0
&tchiness$ especially following a warm bath or shower
Headache
1i22iness
(eaness
E#cessive sweating
Painful swelling of one 3oint$ often the big toe
*hortness of breath
+reathing di4culty when you lie down
5umbness$ tingling$ burning or weaness in your hands$ feet$ arms or legs
6 feeling of fullness or bloating in your left upper abdomen due to an
enlarged spleen
(hen to see a doctor
,ae an appointment with your doctor for an evaluation if you have any of
the signs or symptoms of polycythemia vera!
+ecause polycythemia vera causes your blood to thicen and slows blood
7ow$ it increases your ris of developing blood clots! &f a blood clot occurs in
your head$ it can cause a stroe! *ee emergency medical care if you have
any of the following signs or symptoms of a stroe0
*udden numbness$ weaness$ or paralysis of your face$ arm or leg " usually
on one side of your body
*udden di4culty speaing or understanding speech (aphasia)
*udden blurred$ double or decreased vision
*udden di22iness$ loss of balance or loss of coordination
6 sudden$ severe headache or an unusual headache$ which may or may not
be accompanied by a sti. nec$ facial pain$ pain between your eyes$ vomiting
or altered consciousness
-onfusion$ or problems with memory$ spatial orientation or perception
-auses
+y ,ayo -linic *ta.
Polycythemia vera occurs when a mutation in a bone marrow cell causes a
problem with blood cell production! 5ormally$ your body carefully regulates
the number of each of the three types of blood cells you have! +ut in
polycythemia vera$ the mechanism your body uses to control the production
of blood cells becomes damaged$ and your bone marrow maes too many of
some blood cells!
The mutation that causes polycythemia vera is thought to a.ect a protein
switch that tells the cells to grow! *peci)cally$ it%s a mutation in the protein
869: (the 869: V;<=/ mutation)! ,ost people with polycythemia vera have
this mutation! There are other mutations found in people with polycythemia
vera$ but it%s not yet nown what role these mutations play in the
development of the disease or what the implications of these mutations
might mean for treating the disease!
&t%s not clear what causes the mutations seen in polycythemia vera!
Researchers believe the mutation occurs after conception " meaning that
your mother and father don%t have it " so it%s ac>uired$ rather than inherited
from a parent!
-omplications
+y ,ayo -linic *ta.
Possible complications of polycythemia vera include0
+lood clots! Polycythemia vera causes your blood to be thicer than normal$
which can slow the rate of blood 7ow through your veins and arteries!
&ncreased blood thicness and decreased blood 7ow$ as well as abnormalities
in your platelets$ increase your ris of blood clots! +lood clots can cause a
stroe$ a heart attac$ or blocage of an artery in your lungs (pulmonary
embolism) or in a vein deep within a muscle (deep vein thrombosis)!
Enlarged spleen (splenomegaly)! ?our spleen helps your body )ght infection
and )lter unwanted material$ such as old or damaged blood cells! The
increased number of blood cells caused by polycythemia vera maes your
spleen wor harder than normal$ which causes it to enlarge!
*in problems! Polycythemia vera may cause your sin to itch$ especially
after a warm bath or shower$ or after sleeping in a warm bed! ?ou may
e#perience a burning or tingling sensation in your sin$ particularly on your
arms$ legs$ hands or feet! ?our sin may also appear red$ especially on your
face!
Problems due to high levels of red blood cells! Too many red blood cells can
lead to a number of other complications$ including open sores on the inside
lining of your stomach$ upper small intestine or esophagus (peptic ulcers) and
in7ammation in your 3oints (gout)!
'ther blood disorders! &n rare cases$ polycythemia vera may lead to other
blood diseases$ including a progressive disorder in which bone marrow is
replaced with scar tissue (myelo)brosis)$ a condition in which stem cells don%t
mature or function properly (myelodysplastic syndrome)$ or cancer of the
blood and bone marrow (acute leuemia)!
Tests and diagnosis
+y ,ayo -linic *ta.
6ppointments @ care
6t ,ayo -linic$ we tae the time to listen$ to )nd answers and to provide you
the best care!
Aearn more! Re>uest an appointment!
,ultimedia
&llustration showing a needle suctioning out li>uid bone marrow from
hipbone+one marrow biopsy
+lood tests
1octors most fre>uently use blood tests to diagnose polycythemia vera! &f
you have polycythemia vera$ blood tests may reveal0
6n increase in the number of red blood cells and$ in some cases$ an increase
in platelets or white blood cells!
Elevated hematocrit measurement$ the percentage of red blood cells that
mae up total blood volume!
Elevated levels of hemoglobin$ the iron-rich protein in red blood cells that
carries o#ygen!
Very low levels of erythropoietin$ a hormone that stimulates bone marrow to
produce new red blood cells!
+one marrow aspiration or biopsy
&f your doctor suspects you have polycythemia vera$ he or she may
recommend a bone marrow aspiration or biopsy to collect a sample of your
bone marrow! 6 bone marrow biopsy involves taing a sample of solid bone
marrow material! 6 bone marrow aspiration is usually done at the same time
as a biopsy! 1uring an aspiration$ your doctor withdraws a sample of the
li>uid portion of your marrow!
&f an e#amination of your bone marrow shows that it%s producing higher than
normal numbers of blood cells$ it may be a sign of polycythemia vera!
Tests for the gene mutation that causes polycythemia vera
&f you have polycythemia vera$ analysis of your bone marrow or blood also
may show the mutation in the cells (869: V;<=/ mutation) that%s associated
with the disease
Treatments and drugs
+y ,ayo -linic *ta.
6ppointments @ care
6t ,ayo -linic$ we tae the time to listen$ to )nd answers and to provide you
the best care!
Aearn more! Re>uest an appointment!
Polycythemia vera is a chronic condition that can%t be cured! Treatment
focuses on reducing your amount of blood cells! &n many cases$ treatment
can prevent complications from polycythemia vera and decrease or eliminate
the disease%s signs and symptoms!
Treatment may include0
Taing blood out of your veins! 1rawing a certain amount of blood out of your
veins in a procedure called phlebotomy is usually the )rst treatment option
for people with polycythemia vera! This reduces the number of blood cells
and decreases your blood volume$ maing it easier for your blood to function
properly! How often you need phlebotomy depends on the severity of your
condition!
Aow-dose aspirin! ?our doctor may recommend that you tae a low dose of
aspirin to reduce your ris of blood clots! Aow-dose aspirin may also help
reduce burning pain in your feet or hands!
,edication to decrease blood cells! /or people with polycythemia vera who
aren%t helped by phlebotomy alone$ medications$ such as hydro#yurea
(1ro#ia$ Hydrea)$ to suppress your bone marrow%s ability to produce blood
cells may be used! &nterferon alpha may be used to stimulate your immune
system to )ght the overproduction of red blood cells!
Therapy to reduce itching! &f you have bothersome itching$ your doctor may
prescribe medication$ such as antihistamines$ or recommend ultraviolet light
treatment to relieve your discomfort! ,edications that are normally used to
treat depression$ called selective serotonin reuptae inhibitors (**R&s)$ may
be helpful in relieving itching! E#amples of **R&s include paro#etine (Pa#il) or
7uo#etine (Pro2ac)!
Aifestyle and home remedies
+y ,ayo -linic *ta.
?ou can tae steps to help yourself feel better if you%ve been diagnosed with
polycythemia vera! Try to0
E#ercise! ,oderate e#ercise$ such as waling$ can improve your blood 7ow$
which decreases your ris of blood clots! Aeg and anle stretches and
e#ercises also can improve your blood circulation!
6void tobacco! Bsing tobacco can cause your blood vessels to narrow$
increasing the ris of heart attac or stroe due to blood clots!
+e good to your sin! To reduce itching$ bathe in cool water and pat your sin
dry! 6void hot tubs$ heated whirlpools$ and hot showers or baths! Try not to
scratch$ as it can damage your sin and increase the ris of infection! Bse
lotion to eep your sin moist!
6void e#treme temperatures! Poor blood 7ow increases your ris of in3ury
from hot and cold temperatures! &n cold weather$ always wear warm clothing$
particularly on your hands and feet! &n hot weather$ protect yourself from the
sun and drin plenty of li>uids!
(atch for sores! Poor circulation can mae it di4cult for sores to heal$
particularly on your hands and feet! &nspect your feet regularly and tell your
doctor about any sores!
CCCCCCCCCCCCC
Polycythemia Rubra Vera (PRV)
Presentation: ruddy cyanosis, headache, dyspnea or orthopnea, dizziness,
eye complaints, epigastric discomfort / thrombosis (because of
erythrocytosis, not thrombocytosis) / high Hb, dark, viscous blood /
accompanied by leukocytosis and thrombocytosis (releases K causing
spurious hyperkalemia)
!omplications: "#$ get CML
Diagnosis: %&K" '()*+ ,!-
Treatment:
phlebotomy (can give &.& but no evidence that &.& reduces
incidence of thromboses if asymptomatic and normal Hct )/#*)
chemotherapy for symptomatic splenomegaly (increased risk of
leukemogenesis /ith use of hydro0yurea)
Secondary PRV due to lo/ o0ygen saturation state (lung disease,
intracardiac shunt, hypoventilation, &' malformation, altitude), elevated
carbo0yhemoglobin (smoking, !1 poisoning), high2affinity
hemoglobinopathy, some tumors (-!!, H!!, uterine leiomyoma),
hormonal disorders
high erythropoietin / usually not splenomegaly and immature cells in
peripheral blood