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Preceptor : dr. Hj. Ihsanil Husna Sp.PD

Ibnu Fajar Sidik


• Polycythaemia comes from the Greek language poly

(many), cyt (cell), and hemia (blood).

• Polycythaemia is an increase of red blood cells in the

circulation due to excessive red blood cell formation by
the bone marrow  in increased viscosity and blood

• People with polycythemia have elevated

– hematocrit exceeding 6 million / mm
– hemoglobin exceeding 18 g / dl.

• This disorder is most commonly

found in the 50's.
• Men are exposed a little more than

• As a slow-growing neoplastic disease

• polycythemia occurs because :

– Part of the erythrocyte population comes from an
abnormal parent clone  these abnormal blood
stem cells do not require erythropoetin for its
– In a state with low arteiral oxygen saturation
– Erythropoetin increased nonphysiologically in the
paraneoplastic syndrome
Predisposing factor
abnormalities Abnormal Epo,
mutations in erythropoiesis
of stem cells response EPo-R
Gen JAK 2

JH2 STAT hematopoietic

JAK 2 JAK protein
autoinhibitor aktivation activation
mutation phosphorylation

increased cell
occurs valine mutation -> phenylalanine cycle rate

Pain production of
uric acid
JH2 slightly improvement of
autoinhibitor hematopoietic all production of increased blood
action growth all sorts viscosity

oxygenation disruption of target decrease in

disorders organ oxygenation transport rate

activity inability to and nutrients
intolerance digest food are less than
Polycythemia Secondary Relative
Vera Polycythemia Polycytemia
• Primary means • generally occurs • pseudo-
that in response to polycythemia
polycythemia is other factors or • the result of
not caused by underlying shrinking of
other disorders conditions or plasma volume
• In primary disorders
polycythemia • such as liver
the increase in tumors, kidney
red blood cells is tumors or
due to inherent Cushing's
problems. syndrome.
Clinical symptoms

• Face redness (pletora)

• Hyperviscosity causes decreased blood flow  in tissue
hypoxia with clinical manifestations: headache,
dizziness, vertigo & visual impairment
• Bleeding manifestations : epistaxis, gastrointestinal tract
hemorrhage (peptic ulcer)
• Manifestations of arterial and venous thrombosis:
cerebrovascular disorders, myocardial infarction,
• Hepatomegaly.
• Pruritus urticaria.
• Gout.
Diagnostic examination

• Erythrocytes: Increases> 6 million / mL, and erythrocyte smear is

usually normochrome, normocytic
• Granulocytes: increased in cases of polycythemia,
• Platelets: ranging from 450-800 thousand / mL
• B12 serum: Serum B12 may increase in 35% of cases, but may
decrease in ± 30% of cases,
• Bone marrow examination (SST): unless there is a suspicion of
myeloproliferative disease.
• Increased hemoglobin ranges from 18-24 g / dl.
Diagnostic examination

• Hematocrit increase can reach > 60%.

• Blood viscosity increased 5-8 times normal.
• UBBC (Unsaturated B12 Binding Capacity) increased 75% of patients.
• Cytogenetic examination
• Serum erythropoietin: In polycythemia serum erythropoitin serum
according to or normal whereas in secondary polycythemia serum
eritropoitin increase

• complications such as blood clots, bleeding,

acute myelogenous leukemia, peptic ulcer,
gastrointestinal bleeding, heart attack and
• Flebotomy
– Flebotomy may be the only form of treatment required for many
patients, sometimes for many years and is the recommended treatment.
– Indications of flebotomy are primarily in all patients at the onset of
the disease, and in patients who are still of child-bearing age.
– In the flebotomy, a small amount of blood is taken daily until the
hematocrit values ​begin to decline.

• Cytostatic Chemotherapy / Myelosuppressive Therapy

– The goal of cytostatic chemotherapy treatment is sitoreduction.
– Myelosuppressive therapy may be combined with a flebotomy or
administered as a substitute for flebotomy.
– The recommended chemotherapy is Hydroxyurea

• Radioaktive Phosphorus (P32)

– used as a means of suppressing bone marrow.
– P32 was first given with a dose of about 2-3mCi / m2 intravenously,
when given orally the dose was increased by 25%.

• Biological Chemotherapy (Cytokines)

– to control thrombocythaemia
– Biological products used are Interferon (Intron-A, Roveron-) used
primarily in uncontrolled circumstances of thrombocythemia.

• Polycythaemia is a chronic disease and if without

treatment the patient survives an average of 18
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Thankyou …