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Polisitemia

dr Putra Hendra SpPD


UNIBA

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)

Chronic pulmonary or cardiac disease


Decreased 2,3-diphosphoglycerate
High oxygen affinity hemoglobinopathy
Increased carboxyhemoglobin (in smokers)
and methemoglobin
Residence at high altitude
Adrenal cortical hypersecretion
Hydronephrosis
Tumors producing erythropoietin or
anabolic steroids
Relative (stress)
Disorders associated with decreased
plasma volume (e.g., diarrhea, emesis,
renal diseases)

Diagnosis:

Other Signs and

Symptoms of Polycythemia Vera


More Common
Hematocrit level >52
percent (0.52) in
white men, >47
percent (0.47) in
blacks and women
Hemoglobin level >18
g per dL (180 g per L)
in white men, >16 g
per dL (160 g per L)
in blacks and women)
Plethora
Pruritus after bathing
Splenomegaly
Weight loss
Weakness
Sweating

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

(> than 18 g per dL [180 g per L] in


white men and > than 16 g per dL
[160 g per L] in blacks and women)

hematocrit level greater than 52


percent (0.52) in white men and
47 percent (0.47) in blacks and
women

A diagnosis of polycythemia vera is made when a patent


fulfills
all three of the major criteria
Or
any two major and any two minor criteria
Major Criteria
total RBC vol
Men > or = to 36 mL/kg
Women > or = to 32 mL/kg
arterial 02 saturation > or = to 92%
Splenomegaly
Minor Criteria
Thrombocytosis with platelet count > 400,000/mL
Leukocytosis with WBC > 12,000/mL
Increased leukocyte alkaline phosphatase LAP > 100U/L
(no infection)
Serum B12 > 900 pg/mL or binding capacity UB12 BC >
2200 pg/mL

P vera - symptoms
Sx common to all erythrocytosis

Headache, mental acuity, weakness

Sx more specific to P vera

Pruritis after bathing


Erythromelalgia
Hypermetabolic symptoms
Thrombosis (arterial or venous)
Hemorrhage

P vera - PEx Findings


Facial plethora
Splenomegaly

found in 70% of pts

Hepatomegaly
40% of pts

Distension of retinal veins

P vera - Lab Findings


CBC

Hgb/Hct
WBC in 45%
Plts in 65%
Basophilia (seen in all MPDs)

Uric acid (can lead to gout) and B12


Leukocyte alkaline phosphatase score
Low epo levels
Positive JAK2 V617F

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

support the diagnosis of a


myeloproliferative process.

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

Remove 500 cc blood 1-2x/wk to


target Hct 45%, then maintain

P vera - Myelosuppression
Hydroxyurea

can be used in conjunction with phlebotomy


May increase the risk of leukemic
transformation from 1-2% to 4-5%

32

increase the risk of leukemic


transformation from 1-2% to 11%
May be appropriate for pts intolerant of
medications or for elderly patients
Single injection may control hemoglobin and
platelet count for a year or more.

P vera - interferon alpha

Benefits

No
No
No
OK

myelosuppression
increase in progression to AML
increase in thrombosis risk
in pregnancy

Drawbacks

Must be given by injection


Side effects may be intolerable in
many pts, include flu-like symptoms,
fatigue, fever, myalgias, malaise

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).

Risk stratification in PCV


Risk category

Age > 65 or
h/o
thrombosis

Cardiovascular risk
factors ?
(high cholesterol, DM,
smoking, HTN, CHF)

Recommended treatment

Low

No

No

Phlebotomy and low dose


ASA

Intermediate

No

Yes

Hydrea, phlebotomy and


ASA

High

Yes

Hydrea, phlebotomy and


ASA

Consider IFNa in pregnant women and busulfan or 32P in elderly

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

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