Polycythemia Vera

POLYCYTHEMIA VERA


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.

Exam Important

  • Polycythemia vera is a clonal disorder involving a multipotent hemopoietic progenitor cell resulting in panmyelosis (increase RBC, granulocytes, platelets) in absence of any cause.
  • It has an association with activating point mutation in the tyrosine kinase JAK- 2.

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.
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