Polycythemia Vera / Osler Vaquez disease

• Increased production of RBC, WBC and Platelets.
• Differntiation is normal therefore all the cells produced are normal.

C/F :- these are very imp remember them all
• Hyperuricemia - Increased turnover of cells
• Intense Itching - Due to histamine release (Increase in Basophil causes this)
• Gouty Arthritis - Hyperuricemia
• Peptic Ulcer disease - Histamin & H.pylori
• Erythromelalgia (Burning pain and reddish bluish discoloration of skin) - Increased Platelet aggregation
• Deep Vein Thrombosis - Megakaryocytosis
• Hypertension
• Neurological - Headache, Vertigo, Tinnitus
Erythromelalgia


Erythromelalgia


Diagnosis :-
• Tyrosine Kinase (JAK2) gene mutation
• RBC >6 million/cu mm
• Hematocrit > 0.52 in men > 0.48 on women
• Hb > 18 gm%
• ESR is reduced
• Increased NAP, Increase in transcobalamine
• Increased Platelet count


• Incidence of infection is not Increased

Tumours associated :- Questions are frequently asked from these. Learn them all
1. Hypernephroma
2. Hepatoma
3. Adrenal adenoma
4. Pheochromocytoma
5. Cerebellar Hemangioblastoma
6. Uterine Fibromyoma
7. Meningioma
8. Cushing Syndrome


For more such articles like - Cortex Mednotes

T/t :-
Phlebotomy
Low dose Aspirin (75 mg)
Interferon
Erlotinib

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