Myeloid malignancies Flashcards

1
Q

What is included in myeloid malignancies?

A

AML
CML
Myelodysplastic syndromes (MDS)
Myeloproliferative neoplasms (MPN)

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2
Q

What are clinical features of AML?

A

Bone marrow failure:

  • Anaemia
  • Thrombocytopenia bleeding (purport and mucosal membrane bleeding)
  • Infection due to neutropenia
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3
Q

What are investigations for AML?

A
Blood count and blood film
Bone marrow aspirate
- blasts >20% marrow cell in AML
Cytogenetics from leukaemia blasts
Immunophenotyping of leukaemia blasts
CSF exam
Targeted molecular genetics - gene mutations
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4
Q

What is the treatment for AML?

A
Supportive
Anti-Leukaemia chemotherapy
- daunorubicin & cytosine arabinoside (DA)
- high dose cytosine arabinoside
- Gemtuzumab Ozogamicin
- CPX-351
Allogeneic stem cell transplantation
All-trans retinoid acid (ATRA) and arsenic trioxide (ATO)
Targeted treatment
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5
Q

What are clinical features of CML?

A
Anaemia
Splenomegaly, often massive
Weight loss
Hyperleukostasis
Gout
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6
Q

How can hyperleukostasis present in CML?

A

Fundal haemorrhage
Venous congestion
Altered consciousness
Resp failure

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7
Q

What does a CML blood count look like

A

High platelets
Slight anaemia
High WBC
High neutrophils
High monos, eos, baso
Presence: blasts, promyelocytes, myelocytes, metamyelocytes
Bone marrow and blood cells contain the Philadelphia chromosome t(9;22)

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8
Q

What is a highly sensitive genetic test for CML?

A

Philadelphia chromosome

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9
Q

What is the Philedelphia chromosome?

A

A specific genetic abnormality in chromosome 22 of leukemia cancer cells (particularly chronic myeloid leukemia (CML) cells)

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10
Q

What is the treatment for CML?

A
Tyrosine Kinase Inhibitors (TKIs)
Allogeneic Transplantation (TKI failures)
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11
Q

What is the mode of action of TKIs?

A

Blocks phosphorylation of tyrosine, pathway of CML blocked off

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12
Q

What is a TKI for CML?

A

Imatinib

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13
Q

What are examples of myeloproliferative neoplasms (MPN)?

A
Polycythaemia Vera (PV)
Essential Thrombocythaemia (ET)
Idiopathic Myelofibrosis
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14
Q

In myeloproliferative neoplasms, where does the proliferation concentrate?

A

RBCs and platelets

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15
Q

What is polycythaemia vera?

A

Blood cancer
Bone marrow makes too many RBCs
High hemotocrit

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16
Q

What is the most common gene mutation that causes polycythaemia vera?

17
Q

What are complications of polycythaemia vera?

A

Enlarged spleen
Blood clots
Inflammation
Peptic ulcers

18
Q

What are the clinical features of polycythaemia vera?

A
Headaches
Itch
Vascular occlusion
Thrombosis
TIA/stroke
Splenomegaly
Bad circulation
Red face/plethoric
19
Q

What are lab features of PV?

A

Raised haemoglobin concentration and haematocrit
Raised WCC and platelet count
Raised uric acid
True increase in red cell mass when blood volume measured

20
Q

What is there a risk of if raised uric acid in PV?

21
Q

What is a normal hematocrit?

22
Q

What is the haematocrit?

A

Ratio of the volume of red blood cells to the total volume of blood.

23
Q

What is the treatment of PV?

A

Venesection (bloodletting)
Aspirin
Hydroxcarbamide (HC)/Alpha interferon

24
Q

What is the main aim of PV treatment?

A

Reduce risk of stroke

25
What is natural history of PV?
Stroke - poorly controlled Bone marrow failure from development secondary myelofibrosis Transformation to AML
26
What is essential thrombocythaemia (ET)?
Myeloproliferative disease with raised platelet count
27
What gene mutations can cause ET?
JAK2 | CALR
28
What are the symptoms of ET?
``` Arterial and venous thromboses Digital ischaemia Gout Headache Mild splenomegaly ```
29
What is the treatment for ET?
Aspirin | Hydroxycarbamide/anagrelide
30
What can ET progress to?
Myelofibrosis | AML
31
What is pancytopenia?
Deficiency of all three cellular components of the blood (red cells, white cells, and platelets)