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POLYCYTHEMIA

Preceptor : dr. Hj. Ihsanil Husna Sp.PD

Ibnu Fajar Sidik


Definition

• 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
volume.

• People with polycythemia have elevated


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

• This disorder is most commonly


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

• 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
maturation
– In a state with low arteiral oxygen saturation
– Erythropoetin increased nonphysiologically in the
paraneoplastic syndrome
Predisposing factor
Phatophysiology
DNA
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
action

increased cell
occurs valine mutation -> phenylalanine cycle rate

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

oxygenation disruption of target decrease in


weak
disorders organ oxygenation transport rate

imbalances
activity inability to and nutrients
intolerance digest food are less than
necessity
Classification
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,
Splenomegaly.
• 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
Complication

• complications such as blood clots, bleeding,


acute myelogenous leukemia, peptic ulcer,
gastrointestinal bleeding, heart attack and
stroke.
Therapy
• 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.
Therapy

• 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
Therapy

• 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%.
Therapy

• Biological Chemotherapy (Cytokines)


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

• Polycythaemia is a chronic disease and if without


treatment the patient survives an average of 18
months.
Reference
• Guyton, Arthur C. 2008. Buku Ajar Fisiologi Kedokteran, Edisi 11. Jakarta: Penerbit Buku Kedokteran
EGC
• Harsono. 2003. Spondilitis Tuberkulosa dalam Kapita Selekta Neurologi. Ed. II. Yogyakarta: Gajah Mada
University Press.
• Hydroxyurea in the First Trimester, Am J Perinatol. 2004; 21(3): 135.
• Passamonti ,F, et al , 2003. Polycythemia vera in young patients: a study on the long-term risk of
thrombosis, myelofibrosis and leukemia, Haematologica 88 (1): 13–8.
• Pata, et al, 2004, Polycythemia Vera and Pregnancy : A case Report with The Use of
• Price, Sylvia A. dan Lorraine M. Wilson. 2009. Patofisiologi Konsep-Konsep Klinis Penyakit Edisi 6. EGC
: Jakarta
• Rasjad C. 2003. Spondilitis Tuberkulosa dalam Pengantar Ilmu Bedah Ortopedi. Ed.II. Makassar: Bintang
Lamumpatue.
• Spival J.L Polycythemia vera. Dalam : Fauci As dkk (Eds). Harisso’s Principles of internal medicine
14thed. New York: Mc graw.
• Spivak, Jerry, L. dan Silver, Richard, T., 2008, The revised World Health Organization diagnostic criteria
for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal, The
American Society of Hematology, Blood 112(2): 231-239.
• Stuart, Brian, J. dan Viera, Anthony, J., 2004, Polycythemia Vera (Practical Therapeutics) ,American
Family Physician 69 (9) : 2139-2144.

Thankyou …