Myeloid Malignacy Flashcards
1
Q
What are the clinical features of AML?
A
- Bone marrow failure
- Anaemia
- Thrombocytopenic bleeding (purpura and mucosal membrane bleeding)
- Infection because of neutropenia (predominantly bacterial and fungal)
2
Q
What investigations need done for AML?
A
- FBC and blood film
- Bone marrow aspirate
- Cytogenetics from leukaemic blasts
- Immunophenotyping of leukaemic blasts
- CSF exam if symptoms (in children)
- Targeted molecular genetics
3
Q
How can AML be treated?
A
- Supportive care
- Anti-leukaemic chemotherapy
- Allogeneic stem cell transplantation
- ATRA and arsenic trioxide
- Targeted treatment e.g. midostaurin
4
Q
What are the anti-leukaemic chemotherapy options for AML?
A
- Daunorubicin & cytosine arabinoside (DA)
- High dose cytosine arabinoside
- Gemtuzumab Ozogamicin
- CPX-351
5
Q
What is the clinical presentation of CML?
A
- Anaemia
- Splenomegaly
- Weight loss
- Hyperleukostasis: fundal haemorrhage, venous congestion, altered consciousness and respiratory failure
- Gout
6
Q
What would you see on a FBC in someone with CML?
A
- Raised WCC
- Low haemoglobin
- Increased platelets
- Increased neutrophils
- Increased basophils
7
Q
What are the laboratory features of CML?
A
- High WCC
- High platelet count
- Anaemia
- Blood film shows all stages of white cell differentiation with increased basophils
- Bone marrow is hypercellular
- Bone marrow and blood cells contain the philadelphia chromosome - t(9;22)
8
Q
How can CML be treated?
A
- Tyrosine Kinase Inhibitors: imartinib, dasatinib, nilotinib, busitinib and ponatinib
- Direct inhibitors of BCR-ABL
- Allogeneic transplantation - in TKI failures
9
Q
Name the different myeloproliferative neoplasms
A
- Polycythaemia vera
- Essential thrombocythaemia
- Idiopathic myelofibrosis
10
Q
Which gene is implicated in myeloproliferative neoplasms?
A
JAK2 V617F mutation
11
Q
What are the clinical features of PRV?
A
- Headaches
- Itch
- Vascular occlusion
- Thrombosis
- TIA or stroke
- Splenomegaly
12
Q
What are the laboratory features of PV?
A
- Raised haemolobin conc. and haematocrit
- Tendency to have a raised WCC and platelet count
- Raised uric acid
- A true increase in red cell mass
- Increased neutrophils and basophils
13
Q
What are the treatment options for PRV?
A
- Venesection (blood letting)
- Aspirin
- Hydroxycarbamide/Alpha Interferon
- Ruxolitinib in HC failures with systemic symptoms
14
Q
What are the potential consequences of PRV?
A
- Stroke and other thromboses if poorly controlled
- Bone marrow failure from the development of secondary myelofibrosis
- Transformation to AML
15
Q
What are the features of essential thrombocythaemia?
A
- Raised platelet count
- 50% positive for JAK2 V617F mutations and 25% for the CALR mutation
- Arterial and venous thromboses
- Mild splenomegaly
