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Systems: Haematology + Immunology > Myeloid Malignancies > Flashcards

Myeloid Malignancies Flashcards

1
Q

Myeloid malignancies arise from what cells?

A
  • Red cells
  • Platelets
  • Granulocytes
  • Monocytes
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2
Q

Give examples of myeloid malignancies.

A
  • Acute Myeloid Leukaemia (AML)
  • Chronic Myeloid Leukaemia (CML)
  • Myelodysplastic Syndromes (MDS)
  • Myeloproliferative Neoplasms (MPN)
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3
Q

Acute vs chronic myeloid leukaemia

Leukaemic cells

A

Acute
Do not differentiate

Chronic
Retain ability to differentiate

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4
Q

Acute vs chronic myeloid leukaemia

Bone marrow

A

Acute
Bone marrow failure

Chronic
Proliferation without bone marrow failure

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5
Q

Acute vs chronic myeloid leukaemia

Survival

A

Acute
Rapidly fatal if untreated

Chronic
Survival for a few years historically

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6
Q

Acute vs chronic myeloid leukaemia

Treatment

A

Acute
Potentially curable

Chronic
Long term survival/ possible cures with modern therapy

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7
Q

What are the sub-groups of acute leukaemia?

A
  • Acute Myeloblastic Leukaemia (AML)

- Acute Lymphoblastic Leukaemia (ALL)

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8
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)
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9
Q

What are the essential investigations in AML?

A
  • Blood count and blood film
  • Bone marrow aspirate/ trephine
  • Cytogenetics (Karyotype) from leukaemic blasts
  • Immunophenotyping of leukaemic blasts
  • CSF examination if symptoms
  • Targeted molecular genetics for associated acquired gene mutations (e.g. FLT3, NPM1, IDH 1 & 2)

-Increasing use of NGS myeloid gene panels in AML

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10
Q

How is AML treated?

A
  • Supportive care
  • Anti-leukaemic chemotherapy (to achieve and consolidate remission)
  • Allogeneic stem cell transplantation –to consolidate remission/potential cure
  • All-trans retinoic acid (ATRA) and arsenic trioxide (ATO) in low risk Acute Promyelocytic Leukaemia: ‘Chemo-free’ (high cure rate ~ 90%)
  • Targeted treatment (e.g Midostaurin in FLT3 mutated AML)
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11
Q

What anti-leukemic chemotherapy is used in treatment of AML?

A
  • Daunorubicin & cytosine arabinoside (DA)
  • High dose cytosine arabinoside
  • Gemtuzumab Ozogamicin
  • CPX-351
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12
Q

What new developments have been made in the treatment of AML?

A
  • Targeted antibodies: (Gemtuzumab Ozogamicin anti-CD33 with Calicheomycin (Mylotarg) )
  • Targeted small molecules (Midostaurin, Tyrosine Kinase Inhibitor including inhibiting FLT3)
  • New delivery systems (CPX -351)
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13
Q

How does CML present?

A
  • Anaemia
  • Splenomegaly, often massive
  • Weight loss
  • Hyperleukostasis : Fundal haemorrhage and venous congestion, altered consciousness, respiratory failure.
  • Gout
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14
Q

What are the laboratory features of CML?

A
  • High WCC ( can be very high )
  • 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)
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15
Q

What is the Philadelphia chromosome translocation?

A

t(9;22)

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16
Q

What is the treatment for CML?

A

Tyrosin kinase inhibitors

  • Imatinib (Glivec)-Dasatinib (Sprycel)
  • Nilotinib (Tasigna)
  • Busitinib
  • Ponatinib

Direct inhibitors of BCR-ABL: first line in all patients nowadays
-Allogenic transplantation (few, only in TKI failure)

17
Q

What are myelodysplastic syndromes?

A
  • Acquired clonal disorders of the bone marrow that are commonly seen in old age
  • They are pre-leukemic
18
Q

How do myelodysplastic syndromes present?s

A

Macrocytic anaemia and pancytopenia

19
Q

What is the outcome of myelodysplastic syndromes?

A

Fatal as a result of progression to bone marrow failure or AML

20
Q

What is the treatment for myelodysplastic syndromes?

A

Supportive or a stem cell transplantation for the few young patients

21
Q

Give examples of myeloproliferative neoplasms.

A
  • Polycythaemia Vera (PV)
  • Essential Thrombocythaemia (ET)
  • Idiopathic Myelofibrosis (IM)
22
Q

What genetic mutations are associated with myeloproliferative neoplasms?

A

JAK2V617 mutation in

  • 95% of PV
  • 50% of ET
  • 50% of Myelofibrosis

CALR mutation in 25% of ET

23
Q

What are the clinical features of PV?

A
  • Headaches
  • Itch
  • Vascular occlusion
  • Thrombosis
  • TIA, stroke
  • Splenomegaly
24
Q

What are the laboratory features of PV?

A
  • A raised haemoglobin concentration and haematocrit.
  • A tendency to also have a raised white cell count and platelet count
  • A raised uric acid
  • A true increase in red cell mass when the blood volume is measured
25
Q

What is a typical blood film of PV?

A

Microcytosis with large and abnormal platelets

26
Q

What is the treatment for PV?

A
  • Venesection to keep the haematocrit below 0.45 - men and 0.43 - women
  • Aspirin
  • ? Hydroxcarbamide (HC)
  • ? Ruxolitinib(JAK2 inhibitor) in HC failures with systemic symptoms
27
Q

What is the natural history of PV?

A
  • Stroke and other arterial or venous thromboses if poorly controlled
  • Bone marrow failure from the development of secondary myelofibrosis
  • Transformation to AML
28
Q

What is ET?

A

A myeloproliferative disease with predominant feature of raised platelet count

29
Q

What genetic mutations are associated with ET?

A
  • 50% positive for JAK2V617F mutation

- 25% positive for calreticulin (CALR) mutation

30
Q

How does ET present?

A
  • Symptoms of arterial and venous thrombosis
  • Digital ischaemia
  • Gout
  • Headache
  • Mild splenomegaly
31
Q

How is ET treated?

A
  • Aspirin

- Hydroxycarbamide/ anagrelide

32
Q

What can ET progress to?

A

Mylefibrosis or AML

Systems: Haematology + Immunology (19 decks)

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