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Haematology > 3: Myelodysplastic Syndrome > Flashcards

3: Myelodysplastic Syndrome Flashcards

1
Q

What is myelodysplastic syndrome

A

Neoplastic disorder of haematopoieitc stem cells characterised by quantitative and qualitative abnormalities in all-3 lineages

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2
Q

What is myelodysplastic syndrome

A

Neoplastic disorder of haematopoieitc stem cells characterised by quantitative and qualitative abnormalities in all-3 lineages

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3
Q

Describe incidence of MDS

A

Incidence increases with age

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4
Q

What % of MDS is primary

A

90

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5
Q

What causes secondary MDS

A
  • Radiation
  • Alkylating agents
  • Benzens
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6
Q

What disorder increases risk of secondary MDS

A

Paroxysmal nocturnal haemaglobinuria

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7
Q

How does RBC deficiency manifest clinically

A

Dyspneoa
Pallour
Lethargy

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8
Q

How does thrombocytopenia manifest clinically

A

Bleeding

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9
Q

How does leucopenia manifest clinically

A

Recurrent Infection

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10
Q

How will MDS present on FBC

A
  • Normocytic anaemia
  • Leucopenia
  • Thrombocytopenia
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11
Q

How will MDS present on bone marrow biopsy

A

Hypercellular bone marrow

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12
Q

what is the only curative option for MDS

A

Allogenic stem cell transplant

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13
Q

what can 30% of MDS progress to

A

Acute myeloid leukaemia

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14
Q

What are myeloproliferative neoplasms

A

neoplastic disorder of haematopoieitc stem cells of the myeloid lineage. It is a quantitative disorder

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15
Q

what are four disorders encompassed in MPN

A
  • CML
  • Polycythemia Rubra Vera
  • Primary Myelofibrosis
  • Essential Thrombocytopenia
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16
Q

what mutation is associated with essential thrombocytopenia, primary myelofibrosis and polycythemia ruby vera

A

JAK2

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17
Q

what is primary myelofibrosis

A

MPN leading to bone marrow fibrosis, extra medullary haematopoiesis and massive splenomegaly

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18
Q

what age does primary myelofibrosis occur

A

Incidence increases with age

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19
Q

what mutation is associated with primary myelofibrosis

A

JAK2

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20
Q

how does primary myelofibrosis present clinically

A
  • Massive splenomegaly
  • Lethargy
  • Weight-Loss
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21
Q

what is polycythaemia rubra vera

A

Condition of increased RBC

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22
Q

what must polycythaemia rubra vera be distinguished from

A
  • Relative polycythaemia

- Secondary polycythaemia

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23
Q

what is relative polycythaemia

A

Apparent increase in RBC, due to plasma deficiency

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24
Q

what is secondary polycythaemia

A

Increase in RBC secondary to chronic hypoxia (eg. COPD)

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25
Q

what mutation is associated with polycythaemia rubra vera

A

JAK2

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26
Q

what is the triad of polycythaemia rubra vera

A
  • Neurological changes
  • Visual changes
  • Mucosal bleeding
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27
Q

what dermatological skin is associated with polycythaemia rubra vera

A

Plethora facies

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28
Q

what is the classic feature of polycythaemia vera

A

Pruritus following a hot bath

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29
Q

what is required to diagnose polycythaemia vera

A
  • Raised RBC
  • Low EPO
  • Hypercellular bone marrow
  • JAK2
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30
Q

what are patients with polycythaemia vera at risk of

A
  • Venous thrombosis

- Arterial thrombosis

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31
Q

how is polycythaemia vera managed

A

Venesection

32
Q

what is ruxolitinib

A

JAK2 inhibitor

33
Q

what are risks of polycythaemia vera

A
  • Venous thrombosis: VTE, Mesenteric, Renal vein thrombosis

- Arterial thrombosis: stroke, MI

34
Q

what malignant disorder can polycythaemia vera progress to

A

AML

35
Q

what is essential thrombocytheaemia

A

Increase in platelets

36
Q

what mutation is associated with essential thrombocythaemia

A

JAK2

37
Q

how may patients with essential thrombocytaemia present

A
  • Burning in hands and feet
  • Recurrent gout
  • Bleeding gums
  • Foetal loss
38
Q

why do patients with ET get gum bleeding

A

many of the platelets formed are dysfunctional

39
Q

how is essential thrombocythaemia managed

A

hydroxycarbamide

40
Q

Describe incidence of MDS

A

Incidence increases with age

41
Q

What % of MDS is primary

A

90

42
Q

What causes secondary MDS

A
  • Radiation
  • Alkylating agents
  • Benzens
43
Q

What disorder increases risk of secondary MDS

A

Paroxysmal nocturnal haemaglobinuria

44
Q

How does RBC deficiency manifest clinically

A

Dyspneoa
Pallour
Lethargy

45
Q

How does thrombocytopenia manifest clinically

A

Bleeding

46
Q

How does leucopenia manifest clinically

A

Recurrent Infection

47
Q

How will MDS present on FBC

A
  • Normocytic anaemia
  • Leucopenia
  • Thrombocytopenia
48
Q

How will MDS present on bone marrow biopsy

A

Hypercellular bone marrow

49
Q

what is the only curative option for MDS

A

Allogenic stem cell transplant

50
Q

what can 30% of MDS progress to

A

Acute myeloid leukaemia

51
Q

What are myeloproliferative neoplasms

A

neoplastic disorder of haematopoieitc stem cells of the myeloid lineage. It is a quantitative disorder

52
Q

what are four disorders encompassed in MPN

A
  • CML
  • Polycythemia Rubra Vera
  • Primary Myelofibrosis
  • Essential Thrombocytopenia
53
Q

what mutation is associated with essential thrombocytopenia, primary myelofibrosis and polycythemia ruby vera

A

JAK2

54
Q

what is primary myelofibrosis

A

MPN leading to bone marrow fibrosis, extra medullary haematopoiesis and massive splenomegaly

55
Q

what age does primary myelofibrosis occur

A

Incidence increases with age

56
Q

what mutation is associated with primary myelofibrosis

A

JAK2

57
Q

how does primary myelofibrosis present clinically

A
  • Massive splenomegaly
  • Lethargy
  • Weight-Loss
58
Q

what is polycythaemia rubra vera

A

Condition of increased RBC

59
Q

what must polycythaemia rubra vera be distinguished from

A
  • Relative polycythaemia

- Secondary polycythaemia

60
Q

what is relative polycythaemia

A

Apparent increase in RBC, due to plasma deficiency

61
Q

what is secondary polycythaemia

A

Increase in RBC secondary to chronic hypoxia (eg. COPD)

62
Q

what mutation is associated with polycythaemia rubra vera

A

JAK2

63
Q

what is the triad of polycythaemia rubra vera

A
  • Neurological changes
  • Visual changes
  • Mucosal bleeding
64
Q

what dermatological skin is associated with polycythaemia rubra vera

A

Plethora facies

65
Q

what is the classic feature of polycythaemia vera

A

Pruritus following a hot bath

66
Q

what is required to diagnose polycythaemia vera

A
  • Raised RBC
  • Low EPO
  • Hypercellular bone marrow
  • JAK2
67
Q

what are patients with polycythaemia vera at risk of

A
  • Venous thrombosis

- Arterial thrombosis

68
Q

how is polycythaemia vera managed

A

Venesection

69
Q

what is ruxolitinib

A

JAK2 inhibitor

70
Q

what are risks of polycythaemia vera

A
  • Venous thrombosis: VTE, Mesenteric, Renal vein thrombosis

- Arterial thrombosis: stroke, MI

71
Q

what malignant disorder can polycythaemia vera progress to

A

AML

72
Q

what is essential thrombocytheaemia

A

Increase in platelets

73
Q

what mutation is associated with essential thrombocythaemia

A

JAK2

74
Q

how may patients with essential thrombocytaemia present

A
  • Burning in hands and feet
  • Recurrent gout
  • Bleeding gums
  • Foetal loss
75
Q

why do patients with ET get gum bleeding

A

many of the platelets formed are dysfunctional

76
Q

how is essential thrombocythaemia managed

A

hydroxycarbamide

Haematology (19 decks)

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