DR Medhat MRCP

• Incidence
- Peaks in the sixth decade (50-60s of age) .
• Features
Hyperviscosity (headaches, tinnitus, visual disturbance, cyanosis, joint pain).
Pruritus, typically after a hot bath.
Splenomegaly ± Hepatomegaly.
Hemorrhage (2ndry to `thrombasthenia`).
Plethoric appearance (d.to ↑ histamine from ↑mast cells)
خد بالك Low ESR , low EPO
Hypertension in a third of patients.
• Complications
1) Arterial and venous thromboses e.g Budd-Chiari syndrome.
2) Around 5-15 % progress to myelofibrosis.
3) Around 5-15% develop an acute leukemia.
- Polycythemia Rubra Vera - around 5-15% progress to myelofibrosis
سؤال or AML
• Investigations of PRV :
سؤال PRV) JAK2 mutation (1 st line investigation for
سؤال PRV) Red cell mass (2 nd line investigation for
FBC/film (raised hematocrit; neutrophils, basophils, platelets raised in half of
patients)
Serum ferritin
Serum erythropoietin level.
ESR (low) & Leucocytic alkaline phosphatase (High)
Bone marrow aspirate and trephine.
RFT & LFT
ABG.
Abdominal ultrasound.
Cytogenetic analysis
Erythroid burst-forming unit (BFU-E) culture
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