Myeloid malignancy

Question 1

What are essential investigations for acute leukemia?

Answer 1

Blood count and blood film
Bone marrow aspirate - Blasts >20% of marrow cells in acute leukemia
Cytogenetics from leukaemic blasts
Immunophenotyping of leukaemic blasts
CSF examination if symptoms
Targeted molecular genetics for associated acquired gene mutations

Question 2

What are clinical features of acute myeloid leukaemia?

Answer 2

Bone marrow failure
Anaemia
Thromboycytopenic bleeding
Infection due to neutropenia - bacterial and fungal

Question 3

How is acute myeloid leukaemia treated?

Answer 3

Supportive care
Anti-leukaemic chemotherapy
Allogeneic stem cell transplantation - consolidate remission/potential cure
All-trans retinoic acid and arsenic trioxide in lowe risk promyelocytic leukaemia
Targeted treatment

Question 4

What drugs are used in anti-leukaemic chemotherapy?

Answer 4

Daunorubicin and cytosine arabinoside
High dose cytosine arabinoside
Gemtuzumab ozogamicin
CPX-351

Question 5

What are new developments of chemotherapy?

Answer 5

Targeted antibodies - gemtuzumab ozogamicin: Anti-CD33 with calicheomycin
Targeted small molecules: Midostaurin: tyrosine kinase inhibitor inhibiting FTL3

Question 6

What is the most effective anti leukaemic strategy?

Answer 6

Allogeneic stem cell transplantation - give patient a new immune system - high risk high reward

Question 7

What are features of chronic myeloid leukaemia?

Answer 7

Anaemia
Splenomegaly
Weight loss
Hyperleukostasis - fundal haemorrhage and venous congestion, altered consciousness, resp failure
Gout

Question 8

What are lab features of CML?

Answer 8

High white cells
High platelets
Anaemia
Blood film shows all stages of white cell differentiation with increased basophils
Bone marrow is hypercellular
Bone marrow and blood cells contain philadelphia chromosome

Question 9

How is CML treated?

Answer 9

Tyrosine kinase inhibitors - imatinib, dasatinib, nilotinib
Busitinib
Ponatinib

Question 10

What are different myeloproliferative neoplasms?

Answer 10

Polycythaemia Vera
Essential Thrombocythaemia
Idiopathic myelofibrosis

Question 11

What are clinical features of polycythaemia vera?

Answer 11

Headaches
Itch
Vascular occlusion
Thrombosis
TIA/stroke
Splenomegaly

Question 12

What are lab features of polycythaemia vera?

Answer 12

Raised haemoglobin concentration and haematocrit
Tendency to have raised white cell and platelet
Raised uric acid
True increase in red cell mass when blood volume is measured

Question 13

What is haematocrit?

Answer 13

Proportion of blood made up by packed red cells after test - normally 45%

Question 14

How is Polycythaemia vera treated?

Answer 14

Venesection to keep haematocrit below 0.45 - MAIN TREATMENT
Aspirin
Hydroxcarbamide/alpha interferon

Question 15

What is natural history of polycythaemia vera?

Answer 15

Stroke and other arterial or venous thromboses if poorly controlled
Bone marrow failure from development of secondary myelofibrosis
Transformation to acute myeloid leukaemia

Question 16

What is the predominant feature of essential thrombocythaemia?

Answer 16

Raised platelets

Question 17

What are clinical features of essential thrombocytopenia?

Answer 17

Arterial and venous thromboses
Digital ischaemia
Gout
Headache
Mild splenomegaly

Question 18

How is essential thrombocytopenia treated?

Answer 18

Aspirin

Hydroxycarbamide/anagrelide