Myeloid Malignancy

Question 1

4 types of myeloid malignancy

Answer 1

• Acute myeloid leukaemia (AML)
• Chronic myeloid leukaema (CML)
• Myelodysplastic syndromes (MDS)
• Myeloproliferative diseases (MPD)

Question 2

Describe acute myeloid leukaemia

Answer 2

• Leukaemic cells don’t differentiate
• Bone marraw failure
• Rapidly fatal if untreated
• Potentially curable

Question 3

Describe Chronic myeloid leukaemia

Answer 3

• Leukaemic cells able to differentiate
• Proliferative without bone marrow failure
• Survival for a few years
• Long term survival/possible cures with modern therapy

Question 4

Sub groups of acute leukaemia

Answer 4

• Acute myeloblastic leukaemia (AML)

- Acute lymphoblastic leukaemia (ALL)

Question 5

Clinical features of AML

Answer 5

• Bone marrow failure
• Anaemia
• Thrombocytopenic bleeding (purpura + mucosal bleeding)
• Infection due to neutropenia (mainly bacterial + fungal)

Question 6

Rx of AML

Answer 6

-Support care
-Anti-leukaemic chemotherapy
-Stem cell transplantation (allogenic)
-Targeted antibodies (midostaurin + Myelotarg anti-CD3 with Calicheomycin)
(Tretinoin in APL (acute promyelocytic leukemia) )

Question 7

Presentation of chronic myeloid leukaemia

Answer 7

• Anaemia
• Splenomegaly (often massive)
• Weight loss
• Gout
• Hyperleukostasis (fundal haemorrhage + venous congestion, altered consciousness + resp. failure)

Question 8

Labratory features of CML

Answer 8

• High WCC
• High platelet count
• Anaemia
• Bone marrow is hypercellular
• Blood film = all stages of WC differentiation with increased basophils
• Bone marrow + blood cells contatin the Philadelphia chromosome

Question 9

Rx of chronic myeloid leukaemia

Answer 9

Direct inhibitors of BCR-ABL first line in all patients nowadays

Tyrosine kinase inhibitors

• Imatinib
• Dasatinib
• Nilotinib
• Busitinib
• Ponatinib

Allogenic transplant (few now) (only in TKI failures)

Question 10

What is pancytopenia

Answer 10

Deficiency of all three cellular components of the blood (red cells, white cells, and platelets).

Question 11

How do myelodysplastic syndromes present

Answer 11

• Macrocytic anaemia + pancytopenia

- Commonly seen in old age

Question 12

What are myelodysplastic syndromes

Answer 12

• Acquired clonal disorders of the bone marrow

- They’re pre-leukaemic

Question 13

Prognosis of myelodysplastic syndromes

Answer 13

They’re fatal as a result of progression to bone marrow failur or AML

Question 14

Rx of myelodysplastic syndromes

Answer 14

• Supportive

- Stem cell transplantation for the few young people

Question 15

3 myeloproliferative diseases

Answer 15

• Polycythaemia vera
• Essential thrombocythaemia
• Idiopathic myelofibrosis

Question 16

Polycythaemia vera clinical features

Answer 16

• Headaches
• Itch
• Vascular occlusion
• Thrombosis
• TIA/Stroke
• Splenomegaly

Question 17

Laboratory features of polycythaemia vera

Answer 17

• Raised Hb conc. + haematocrit
• May have raised WC + platelet count
• Raised uric acid
• Increase in RBC mass when the volume is measured

Question 18

Rx for polycythaemia vera

Answer 18

• Venesection to keep haematocrit below 0.45 in men + 0.43 in women
• Aspirin
• Hydroxcarbamide

Question 19

Natural Hx of polycythaemia vera

Answer 19

• Stroke + other arterial/venous thromboses if poorly controlled
• Bone marrow failure from the development of secondary myelofibrosis
• Transformation to AML

Question 20

What is essential thrombocythaemia

Answer 20

• Myeloproliferative disease, predominant feature is raised platelets
• 5% +ve for JAK2V617F mutation

Question 21

Symptoms of essential thrombocythaemia

Answer 21

• Arterial + venous thromboses
• Digital ischaemia
• Gout
• Mild splenomegaly

Question 22

Rx for essential thrombocythaemia

Answer 22

• Aspirin

- Hydroxyurea

Question 23

What can essential thrombocythaemia progress to

Answer 23

• Myelofibrosis

- AML