Chronic myeloproliferative disorders and chronic myeloid leukaemia

Question 1

Polycythaemia Vera

Answer 1

Increased red cells (and neutrophils and maybe platelets). Can be caused due to altitude.
Diagnosed by looking at pack cell volume: more than 50% rbc is this condition.
Peak at 50-70 years
Symptoms: itching , plethoric face, malaise, gangrene of toes.
Signs: splenomegaly
Treatment: venesections ( take blood frequently)

Question 2

Essential thrombocythaemia

Answer 2

Increased platelets (secondary is following surgery/malignancy/Fe deficiency etc)
Treatment: antiplatelet and asparin needed.
cytoreduction eg by hydroxycarbamide
Tests: Full blood count. can be caused by CalR and jak2 mutation.

Question 3

Myelofibrosis

Answer 3

Variable cytopenias with a large spleen.
Diagnosed with bone marrow results- JAK2 mutation and CalR
Presentation: Pancytopenia (deficiency of all types of blood cells)
Treatment: blood and patelet transfusion, JAK2 inibitors and bone marrow transplant.

Question 4

Chronic myeloid leukaemia

Answer 4

Leukocytosis (increase in the number of white cells) leucoerythroblastic blood picture (the circulating blood contains immature cells of the granulocytic series)
Anaemia, splenomegaly
Symptoms: abdo pain, fatigue, catabolic state, DVT.
Treatment: chemotherapy –> low dose cyctotoxic drugs.
“philadelphia chromosome: 9;22 translocation.
Gleevec = tyrosine kinase inhibitor.