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Polycythemia

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Polycythemia: Types

1.Polycythemia Rubra vera


Accelerated prolofiration of marrow
stem cell
→ ↑ production of RBCs
→ ↑ production of WBCs
→ ↑ production of platelets
2.Secondary Polycythemia
Secondary to other factor
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Polycythemia Vera : Defination

Polycythemia vera is an abnormal

increase in blood cells (primarily red

blood cells) due to excess production of

the cells by the bone marrow


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Polycythemia Vera : Defination

•Increase in the total red cell mass


>36ml/kg in males
>32 ml/kg in females
• It is usually associated with
↑ in HB
↑ in hematocrite
↑ in red cell count
Polycythemia Vera

Age: usually above 40


Sex: males > females
Etiology: Unknown
Usually gradual presentation by
vague symptoms

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Pathophysiology

+++ viscosity of blood


→ risk of thrombosis
→ disturbance of O2 transport
→ peripheral V.D
→ Stagnation of blood in peripheral
capillaries
→ ++ cardiac output
→ cardiomegally &heart failure
Polycythemia Vera: Clinical presentation

•Red coloration, especially of the face (Plethora)

•Dizziness

•Headache

•Symptoms caused by splenomegally (75%)

•Thrombosis

•Hypertention (20-50%)

•Vision problems www.MansFans.com


Polycythemia Vera: Diagnosis

Blood Picture:
RBCs usually () 7-10x106
HB 17-22 gm%
Ht usually () 60-70%
Platelets usually ↑, may reach (million or more)
WBCs ↑ in the majority of cases
Polycythemia Vera: Diagnosis

Bone Marrow Aspiration:


Hyperactivity of all blood elements with ↑
megakaryocytes
Serum Uric acid: increases
E.S.R: Decrease
Erythropoietin Decrease
Polycythemia Vera: Complications

• Myelosclerosis

• Myeloid leukemia

• Vascular thrombosis

• Haemorrhage

• 2ry gout
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Polycythemia Vera:Treatment

• Repeated Venesection:

300-500 c.c each time

repeated according to severity

→ No effect on platelets or WBCs

→ Iron defeciency
Polycythemia Vera:Treatment
2. Radioactive phosphorus:

Long remissions

may lead to leukemia

3. Chemotherapy

Busulphan (Myleran)

Chlorumbucil (leukeran)

Hydroxyurea (Hydrea)
Secondary polycythemia
Physiological:
New born
High altitude
Pathological
Obstructive lung disease
congenital cyanotic heart disease
Renal tumors
cushing syndrome
Normal WBCs - Platelets- no splenomegally

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