Categories
Internal Medicine

Polycythaemia Vera (Polycythaemia Rubra Vera)

Last Updated on October 13, 2022

Introduction

• A myeloproliferative disorder caused by malignant proliferation of a clone derived from one pluripotent stem cell leading to an excess proliferation of red blood cell, leucocytes and platelets → hyperviscosity of blood & thrombosis
JAK2 mutation is present in >95% cases
• Age group: commoner >60s, peak in 60s

Presentation

(i) Asymptomatic
• Detected from FBC

(ii) Symptomatic

Clinical Features

Symptom

Vague symptoms
• e.g. headache, tinnitus, visual disturbance

Characteristic symptoms
• Body itching, typically after a hot bath
• Erythromelalgia — burning sensation in fingers and toes

Physical examination

• Plethoric appearance
• Splenomegaly

Other features

• Patient may have features of arterial thrombosis or venous thrombosis
Hypertension
• Low ESR

Investigations

Blood test

FBC
• Serum ferritin
• ABG
Liver function test
• Renal profile
JAK2

Imaging

• Ultrasound abdomen

Others

• Bone marrow aspirate and trephine (BMAT)

Complications

• Thrombosis
Haemorrhage

Management

Treatment options

• Tab Aspirin 75 mg OD
Venesection
Chemotherapy

Target

• Keep haematocrit less than 45%

Prognosis

• Variable; many remain well for years
• Thrombotic events may cause significant morbidity & mortality
• 30% will develop myelofibrosis
• 5% will develop acute leukaemia

Leave a Reply

Your email address will not be published. Required fields are marked *

error: Content is protected !!