POLYCYTHEMIA VERA

• Polycythemia vera is a clonal disorder involving a multipotent hemopoietic progenitor cell resulting in panmyelosis (increase RBC, granulocytes, platelets) in absence of any cause.
• More common in females.

 Etiology-

• It has an association with activating point mutation in the tyrosine kinase JAK- 2.

Types-

1. Primary polycythemia-

• It is the most common of all myeloproliferative disorders.

2. Secondary polycythemia or erythrocyotis

Clinical features-

• Hyperviscosity, hypovolaemia, hypermetabolism, erythocytosis, thrombosis.
• Headache, vertigo, tinnitus, syncope or even coma, transient visual loss
• Splenomegaly, haemtemesis and melena, bleeding.
• Pruritis & peptic ulceration (basophilia with histamine release)
• Hyperuricaemia- urate stones and gout

Diagnosis-

• Raised Hb
• Erythrocytosis
• Leucocytosis
• Thrombocytosis, increased Vitamin B12
• Panhyperplasia
• Cytogenic abnormalities such as 20q, trisomy 8 and 9q
• High hemocrit and JAK-2 mutation usually makes the diagnosis
• Increase erythropoietin

Morphological features-

• Basophilia & abnormal large platelets are seen on peripheral blood
• 2% of patients transform to AML.

Treatment-

• Phelbotomy
• Anticoagulant therapy
• Chemotherapy
• Uricosuric drugs treat hyperuricaemia
• Inteferon- α reduces JAK- 2 expression.

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