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13 Cards in this Set

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Gaisbock polycythemia

Polycythemia due to stress

What cell lineages are proliferated?

Erythroid


Myeloids


Megakaryocytes (platelets)


Granulocytes

Types of polycythemia

Absolute-- primary and secondary


Relative

5 causes of absolute polycythemia

Primary polycythemia


Hypoxia


Carboxyhaemoglobim


Cushing syndrome or steroids


Erythropoietin secreting tumours

Epidemiology of polycythemia

Diagnosis is at age 60yrs


Occurs in 3 of 100,000


Rare below age 30yrs

Complications due to thrombosis in PCV

Cardiovascular accident


Transient Ischaemic Attack


Myocardial infarction


Deep vein thrombosis

Physical findings in pcv

Splenomegaly


Hepatomegaly


Face plethora

Symptoms due to hyperviscosity

Dizziness


Weakness


Headache


Priapism


Paraesthesia

Diagnostic criteria of pcv - minor

Hypercellular of BM


Low EPO


Endogenous erythroid colony growth (EEC)

Diagnostic criteria- Major

In men: Hb >18.5


HCT> 0.52



In women: Hb> 16.5


HCT> 0.48



Presence of JAK2-V617F mutation

Rx of PCV

Phlebotomy


Hydroxyurea


Alpha interferons


Busulphan


Radioactive phosphorus

Causes of secondary thrombocytosis

Malignancies


Infections


Post surgical status


Connective tissue disorders


Iron deficiency anaemia


BM recovery due to alcohol and smoking


Splenectomy

Prognosis

Without therapy survival rate is 6-18monthe


With therapy it is > 10yrs


Up to 20% of pts develop AML or MDS


haemorrhage and thrombosis are the major cause of DEATH