Myeloid Malignancies Flashcards Preview

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Flashcards in Myeloid Malignancies Deck (23)
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1

What are the 4 major myeloid malignancies?

AML
CML
Myelodysplastic Syndromes
Myeloproliferative Neoplasms

2

How does AML work?

A mutation in a stem cell --> Leukaemic stem cell

Allows self-renewal of progenitor cells, proliferation & blocking of differentiation

Leads to a mass of blast cells pushing out the healthy stem cells --> Bone marrow failure

3

Clinically how would you encounter a case of AML?

Bone Marrow Failure:
- Anaemia
- Thrombocytopenic bleeds (Purpura/petechiae & mucosal bleeds)
- Neutropenic infections (bacterial/fungal)

4

How would you approach an AML patient?

FBC & blood film
Bone Marrow Aspirate (confirms)

5

Once you have a diagnosis of leukaemia what further testing is done?

Cytogenetics & immunophenotyping of blasts (allows determining myeloblasts vs lymphoblasts as well as subclasses)

CSF exam if they have CNS symptoms

Targeted molecular genetics - Assesses ass mutations that tell us about prognosis and treatment e.g. FLT3

6

How do we manage someone with AML?

- Supportive Care
- Anti-leukemic Chemo
- Allogenic Stem Cell Transplant


Targeted Treatment e.g. Midostaurin in FLT3 AML
Monoclonal Antibodies

7

How can you treat Low risk Acute Promyelocytic Leukaemia?

Can do it "chemo-free" with:
ATRA (all-trans retinoic acid) & ATO (Arsenic Trioxide)

8

How would a patient with CML present?

Anaemia
Splenomegaly
Weight loss
Gout
Hyperleukostasis

9

What are the presentations of hyperleukostasis?

Fundal Haemorrhage
Venous Congestion
Altered Congestion
Resp Failure

10

How do we test for CML?

FBC & Blood Film (High WCC, platelets, myeloid cells & anaemia)

Bone Marrow Aspirate - Hypercellular

Philadelphia Chromosome t(9;22) found in bone marrow & blood cells

11

How can you treat CML?

Not chemo:
- Tyrosine Kinase inhibitors e.g. Imatinib (Glivec)
- Allogenic Stem Cell Transplants (If TKI fails)

12

What are myelodysplastic syndromes?

Acquired clonal disorders of the bone marrow that mostly affect elderly people

13

How do myelodysplastic syndromes present?

With macrocytic anaemia & pancytopenia

It can progress to bone marrow failure & AML

14

How do you treat Myelodysplastic syndromes?

Not curative
Treatment is supportive
Some young patients can get stem cell transplant

15

What are the major types of myeloproliferative neoplasms?

Polycythaemic Vera (PV)
Essential Thrombocythaemia (ET)
Idiopathic Myelofibrosis (IM)

16

What mutations are associated with Myeloproliferative neoplasms?

JAK2V617F - 95% of PV & 50% of ET

CALR - 25% of ET

17

What happens in PV?

Excess of myeloid cells (predominantly RCs) -->
Headache
Itch
Vascular occlusion & thrombosis
Splenomegaly

18

What test results would you expect in PV?

FBC:
- Raised Hb & haematocrit
- Raised WCC & platelets
- Raised URic Acid
- Increased RC mass

19

How do you treat a case of PV?

Venesection (lowers haematocrit)
Aspirin

Hydroxycarbamide --> IF fails use Ruxolitinib (JAK2 inhibitor)

20

When would we use Hydroxycarbamide in PV cases?

Only if they have additional vascular risk factors e.g. HTN or DM
OR if they're >60

21

What is Essential Thrombocythaemia?

A myeloproliferative neoplasm, predominantly affecting platelets

- Thrombi
- Digital Ischaemia
- Gout
- Headache
- Splenomegaly

22

How do we treat ET?

Aspirin
Hydroxycarbamide or Anagrelide

23

What's the danger of Myeloproliferative Neoplasms?

They can progress to Myelofibrosis (causes bone marrow failure) or AML