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Haematology > Myeloid Malignancy > Flashcards

Myeloid Malignancy Flashcards

1
Q

What is acute myeloid leukaemia?

A

Haematological malignancy resulting in the overproduction of immature myeloid cells/myeloid progenitors, building up and replacing bone marrow, causing bone marrow failure

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2
Q

What age group is acute myeloud leukemia more common?

A

Peak age of onset 70 years

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3
Q

How common is AML?

A

The more common form of acute leukaemia in adults

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4
Q

What is the prognosis of AML?

A

Long term survival 50%

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5
Q

Give risk factors for AML

A

Down Syndrome

Radiotherapy

Chemotherapy

Secondary transformation of a myeloproliferative disorder

Associated conditions

  • Chronic myeloid leukaemia
  • Myelodysplasia
  • Myelofibrosis
  • Polycythaemia rubra vera
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6
Q

How does AML present?

A

Anaemia

  • Dyspnoea
  • Fatigue
  • Malaise/weakness
  • Pallor

Thrombocytopenic bleeding

  • Purpura and muscle membrane bleeding

Infection

  • Prolonged neutropenia

Bone pain and tenderness

Gum swelling

Lymphadenopathy

Splenomegaly

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7
Q

When does gum swelling occur in AML?

A

Only in monocytic variety, due to monocytic infiltration

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8
Q

What is an ecchymosis?

A

Large bruise

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9
Q

What investigations are used in AML diagnosis?

A

Bone marrow biopsy

  • Auer rods
  • High numbers of blast cells

FBC

  • Anaemia
  • Increased WCC
  • Thrombocytopenia

Blood film

  • Leukaemic myeloblasts within blood
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10
Q

What biopsy sign is seen in AML?

A

Blasts >20% of marrow cells

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11
Q

How is AML managed?

A

Supportive

  • Anti-fungal and antibiotic prophylaxis
  • Blood transfusions

Chemotherapy

Allogenic stem cell transplantation

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12
Q

What classifications is used for AML?

A

French-American-British (FAB)

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13
Q

What is acute promyelocytic leukaemia M3?

A

Type of AML associated with t(15;17) and the fusion of PML and RAR-alpha genes. It presents younger and is associated with

  • Auer rods (seen with myeloperoxidase stain)
  • DIC or thrombocytopenia at presentation
  • Good prognosis
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14
Q

Who is a allogenic stem cell transplant usually from and why?

A

Usually from a sibling, their immune system recognises the leukemic cells as an antigen

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15
Q

Give complications of AML

A

Relapse

Severe bleeding and infection

CNS infiltration

DIC

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16
Q

What are the poor prognostic features of AML?

A

Over 60 years

Over 20% blasts after first course of chemo

Cytogenetics

  • Deletions of chromosome 5 or 7
17
Q

What is chronic myeloid leukaemia?

A

Malignancy of granulocytes leading to increased production of myeloid precursors, with their differentiation ability still intact

18
Q

What chromosome is associated with CML?

A

Philadelphia chromosome

(Philadelphia cheese makes lunch)

19
Q

What is the Philadelphia chromosome?

A

Reciprocal translocation of chromosome 9 and 22, causing the BCR/ABL fusion gene

20
Q

What activity is associated with the Philadelphia chromosome?

A

Uncontrolled tyrosine kinase activity

21
Q

What age group is CML more common in?

A

Occurs in middle aged and elderly

22
Q

How does CML present?

A

Anaemia

Massive splenomegaly due to build up of myeloid cells

Weight loss

Gout

Sweating

Bleeding

Bruising

Petechiae

Hyperleukostasis

23
Q

What is Hyperleukostasis?

A

Significantly increased blast cells, causing

  • Fundal haemorrhage and venous congestion
  • Altered consciousness
  • Respiratory failure
24
Q

What investigations are used in CML diagnosis?

A

Bone marrow biopsy

Philadelphia chromosome

FBC

  • Increased WCC
  • Low lymphocytes but high neutrophils
  • Thrombocytosis
  • Anaemia

Blood Film

  • Granulocytes at different stages of maturation/myeloblasts and granulocytosis

Decreased leukocyte alkaline phosphatase

25
Q

How is CML managed?

A

Supportive

  • Blood transfusions
  • Antibiotics

Tyrosine Kinase Inhibitors

  • Imatinib

Interferon alpha

Allogenic Transplant in TKI failures

26
Q

Give an example of a tyrosine kinase inhibitor

A

Imatinib

27
Q

Name the myeloproliferative disorders

A

Essential thrombocytosis

Polycythaemia rubra vera

Myelofibrosis

28
Q

What are the features of essential thrombocytosis?

A

Platelet count > 600 * 109/l

Both thrombosis (venous or arterial) and haemorrhage can be seen

Characteristic symptom is a burning sensation in the hands

JAK2 mutation is found in around 50% of patients

29
Q

How is essential thrombocytosis managed?

A

Hydroxyurea (hydroxycarbamide) to reduce platelet count

interferon alpha is also used in younger patients

Low-dose aspirin may be used to reduce the thrombotic risk

30
Q

What is polycythaemia vera?

A

Myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets

31
Q

What are the features of polycythaemia vera?

A

Hyperviscosity

Pruritus, typically after a hot bath

Splenomegaly

Haemorrhage/stroke, secondary to abnormal platelet function

Plethoric appearance

HTN

Headaches, dizzy spells and tinnitus

32
Q

What investigations are used in polycythaemia vera?

A

FBC

  • Increased RBC
  • Increased haematocrit
  • Normal EPO

Low ESR

JAK2

33
Q

How is polycythaemia vera managed?

A

Aspirin

Venesection

hydroxyurea, causes slight increased risk of secondary leukaemia

Phosphorus-32 therapy

34
Q

What investigations are used in myelofibrosis?

A

Blood film

  • Tear drop poikilocytes

FBC

  • Anaemia
  • Thrombocytopenia and leucopenia in progressive disease

Haematology (7 decks)

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