Myeloid Malignancies Flashcards

1
Q

What are the 4 major myeloid malignancies?

A

AML
CML
Myelodysplastic Syndromes
Myeloproliferative Neoplasms

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2
Q

How does AML work?

A

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

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3
Q

Clinically how would you encounter a case of AML?

A

Bone Marrow Failure:

  • Anaemia- fatigue
  • Thrombocytopenia leading to Thrombocytopenic bleeds (Purpura/petechiae &a mucosal bleeds)
  • Leukopenia leading to Neutropenic infections (bacterial/fungal)
  • Bone pain and tenderness due to increased cell production
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4
Q

How would you approach an AML patient?

A

1-FBC: depleted healthy cells
2- Blood film
3- Bone Marrow Aspirate (confirms)
- >20% blast cells

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5
Q

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

A

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

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6
Q

How do we manage someone with AML?

A
  • Supportive Care
  • Anti-leukemic Chemo
  • Allogenic Stem Cell Transplan

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

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7
Q

How can you treat Low risk Acute Promyelocytic Leukaemia?

A

Can do it “chemo-free” with:

ATRA (all-trans retinoic acid) & ATO (Arsenic Trioxide)

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8
Q

How would a patient with CML present?

A
Anaemia
Splenomegaly
Weight loss
Gout
Hyperleukostasis
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9
Q

What are the presentations of hyperleukostasis?

A

Fundal Haemorrhage
Venous Congestion
Altered Congestion
Resp Failure

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10
Q

How do we test for CML?

A

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

Bone Marrow Aspirate - Hypercellular

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

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11
Q

How can you treat CML?

A

Not chemo:

  • Tyrosine Kinase inhibitors e.g. Imatinib (Glivec)
  • Allogenic Stem Cell Transplants (If TKI fails)
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12
Q

What are myelodysplastic syndromes?

A

Acquired clonal disorders which are caused by bone marrow cells not maturing properly of the bone marrow that mostly affect elderly people and

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13
Q

How do myelodysplastic syndromes present?

A

With macrocytic anaemia & pancytopenia

It can progress to bone marrow failure & AML

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14
Q

How do you treat Myelodysplastic syndromes?

A

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

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15
Q

What are the major types of myeloproliferative neoplasms?

A
Polycythaemic Vera (PV)
Essential Thrombocythaemia (ET)
Idiopathic Myelofibrosis (IM)
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16
Q

What mutations are associated with Myeloproliferative neoplasms?

A

JAK2 V617F - 95% of PV & 50% of ET

CALR - 25% of ET

17
Q

What happens in PV?

A

Excess of myeloid cells (predominantly RCs)

18
Q

What test results would you expect in PV?

A

FBC:

  • Raised Hb & haematocrit
  • Raised WCC & platelets
  • Raised URic Acid
  • Increased RC mass
19
Q

How do you treat a case of PV?

A

Venesection (lowers haematocrit)
Aspirin

Hydroxycarbamide –> IF fails use Ruxolitinib (JAK2 inhibitor)

20
Q

When would we use Hydroxycarbamide in PV cases?

A

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

21
Q

What is Essential Thrombocythaemia?

A

A myeloproliferative neoplasm, predominantly affecting platelets

  • Thrombi
  • Digital Ischaemia
  • Gout
  • Headache
  • Splenomegaly
22
Q

How do we treat ET?

A

Aspirin

Hydroxycarbamide or Anagrelide

23
Q

What’s the danger of Myeloproliferative Neoplasms?

A

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

24
Q

what is Hyperleukostasis?

A

Increased number of blast cells

25
Q

Presentation of PV?

A
  • Hyperviscosity: Headaches/ dizziness/ tinnitus/ visual disturbance
  • Itch/ burning in fingers/ toes
  • Facial plethora
  • Thrombosis
  • TIA/stroke
  • Splenomegaly