Haematological malignancies Flashcards

1
Q

How do we classify haematological malignancy?

A
  • Myeloid
    • Acute
      • AML
    • Chronic (Myeloproliferative)
      • CML
      • Myelofibrosis
      • Polycythemia Vera
      • Essential thrombocytosis
  • Lyphoid
    • ALL
    • CCL
    • Lymphoma
    • Myeloma
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2
Q

What is the difference between acute myeloid leukaemia and chronic myeloid leukaemia?

A

Acute myeloid leukaemia affects more immature cells.
Chronic myeloid leukaemia affects more mature cells.

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3
Q

Clinical features of haematological malignancy

A
  • Bone marrow failure
    • Anaemia
    • Fatigue
    • Pallor
    • Thrombocytopaenia
      • Bleeding: bruising and epistaxis
    • Dysfunctional white cells
      • Recurrent infections
  • Constitutional symptoms
    • Weight loss
    • Fatigue
    • Fever
    • Loss of appetite
  • Infiltration
    • Hepatosplenomegaly
    • Lymphadenopathy
    • Gum hyperplasia (AML)
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4
Q

General investigations for haematological malignancy

A
  • Bedside
    • Full set of observations Bloods
  • Bloods
    • ​FBC
    • Blood film
    • Clotting screen
  • Imaging
    • CT imaging
  • Special tests
    • Bone marrow aspirate and biopsy
    • Lymph node biopsy
    • Immunophenotype
    • Genetic testing
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5
Q

Definition of acute myeloid leukaemia

A

Proliferation of immature myeloid cells

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6
Q

Epidemiology of acute myeloid leakaemia

A

Most common acute leukaemia in adults

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7
Q

Risk factors for acute myeloid leukaemia

A
  • Age: average age of diagnosis is 68 years old
  • Myelodysplasia: precursor lesion and evolves to AML in 30% of cases
  • Chemotherapy
  • Radiotherapy
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8
Q

Which subtype of acute myeloid leukaemia is associated with t(15;17)?

A

Acute promyelocytic leukaemia (M3)

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9
Q

Which subtype of AML is most commonly associated with gum infiltration?

A

Acute monocytic leukaemia (M5)

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10
Q

Which subtype of acute myeloid leukaemia is associated with disseminated intravascular coagulation?

A

Acute promyelocytic leukaemia

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11
Q

How do you differentiate myelodysplasia (precursor lesion to AML) from AML?

A

In myelodysplasia blast cells are <20% on bone marrow

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12
Q

Investigations in acute myeloid leukaemia

A
  • FBC: leukocytosis, thrombocytopenia, anaemia
    • Blast cells crowd out bone marrow causing low PLTs and Hb
  • Blood film: immature myeloid cells with auer rods
  • Clotting screen: deranged in disseminated intravascular coagulation
  • Bone marrow biopsy: ≥20% myeloblasts is diagnostic
  • Cytogenetic studies: t(15;17)
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13
Q

Management of acute myeloid leukaemia

A
  • Induction
    • APML: all-trans retinoic acid
  • Consolidation
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14
Q

Which translocation is associated with chronic myeloid leukaemia?

A

t(9;22)

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15
Q

Investigations for chronic myeloid leukaemia

A
  • FBC: leucocytosis, thrombocytosis or thrombocytopenia, anaemia
    • Raised granulocyte count
  • Blood film: increased number of mature granulocytes (<10% blast cells)
  • Bone marrow biopsy: increased number of mature granulocytes
    • Myeloblasts not prevalent
  • Cytogenic studies:Philidelphia chromosome t(9;22)
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16
Q

Managment of chronic myeloid leukaemia

A

Tyrosine kinase inhibitor eg imatinib

17
Q

What is blast transformation?

A

Poorly controlled chronic myeloid leukaemia can transform or acute leukaemia.

There will be ≥20% blast cells in blood

2/3rds AML; 1/3rd ALL

18
Q

Definition of myelofibrosis

A

Myeloproliferative condition. Neoplastic proliferation of mature myeloid cells, particularly megakaryocytes, leading to marrow fibrosis.

19
Q

Investigations for myelofibrosis

A
  • FBC: anaemia, other cell counts may be low or variable
  • Blood film: leucoerythroblastic smear
    • Tear drop RBCs, nuculated RBCs, immature granulocytes
  • Bone marrow aspirate:‘dry tap’
  • Bone marrow biopsy: marrow fibrosis
  • Genetics: JAK2 mutation in 50-60% of patients
20
Q

Definition of polycythaemia vera

A

Neoplastic proliferation of mature myeloid cells, particularly RBCs. Can also have thrombocytosis and granulocytosis.

21
Q

Clinical features of polycythaemia vera

A
22
Q

Investigations in polycythaemia Vera

A
  • FBC: raised Hb and haematocrit
    • Granulocytes and PLTs may also be raised
  • EPO: low in polycythaemia vera
    • Raised in secondary polycythaemia eg hypoxia
  • Genetics: JAK2 V617F mutation in 95% of patients
23
Q

Managment of polycythaemia Vera

A
  • Phlebotomy: aim for haematocrit <45%
  • Aspirin
  • Hydroxyurea: in patients at high risk of thrombosis eg >60 years of age
24
Q

Define essential thrombocytosis

A

Neoplastic proliferation of mature myeloid cells, particularly megakarocytes.

Increased risk of bleeding and/or thrombosis.

25
Q

Investigations for essential thrombocytosis

A
  • FBC: thrombocytosis (PLTs > 600,000)
  • Bone marrow biopsy: increased megakaryocytes
  • Genetics: JAK2 mutation in 50-60% of patients
26
Q

Managment of essential thrombocytosis

A
  • Antiplatelet eg aspirin
  • Hydroxycarbamide