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Haematology > 3: Myelodysplastic Syndrome > Flashcards

3: Myelodysplastic Syndrome Flashcards

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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
what mutation is associated with polycythaemia rubra vera
JAK2
26
what is the triad of polycythaemia rubra vera
- Neurological changes - Visual changes - Mucosal bleeding
27
what dermatological skin is associated with polycythaemia rubra vera
Plethora facies
28
what is the classic feature of polycythaemia vera
Pruritus following a hot bath
29
what is required to diagnose polycythaemia vera
- Raised RBC - Low EPO - Hypercellular bone marrow - JAK2
30
what are patients with polycythaemia vera at risk of
- Venous thrombosis | - Arterial thrombosis
31
how is polycythaemia vera managed
Venesection
32
what is ruxolitinib
JAK2 inhibitor
33
what are risks of polycythaemia vera
- Venous thrombosis: VTE, Mesenteric, Renal vein thrombosis | - Arterial thrombosis: stroke, MI
34
what malignant disorder can polycythaemia vera progress to
AML
35
what is essential thrombocytheaemia
Increase in platelets
36
what mutation is associated with essential thrombocythaemia
JAK2
37
how may patients with essential thrombocytaemia present
- Burning in hands and feet - Recurrent gout - Bleeding gums - Foetal loss
38
why do patients with ET get gum bleeding
many of the platelets formed are dysfunctional
39
how is essential thrombocythaemia managed
hydroxycarbamide
40
Describe incidence of MDS
Incidence increases with age
41
What % of MDS is primary
90
42
What causes secondary MDS
- Radiation - Alkylating agents - Benzens
43
What disorder increases risk of secondary MDS
Paroxysmal nocturnal haemaglobinuria
44
How does RBC deficiency manifest clinically
Dyspneoa Pallour Lethargy
45
How does thrombocytopenia manifest clinically
Bleeding
46
How does leucopenia manifest clinically
Recurrent Infection
47
How will MDS present on FBC
- Normocytic anaemia - Leucopenia - Thrombocytopenia
48
How will MDS present on bone marrow biopsy
Hypercellular bone marrow
49
what is the only curative option for MDS
Allogenic stem cell transplant
50
what can 30% of MDS progress to
Acute myeloid leukaemia
51
What are myeloproliferative neoplasms
neoplastic disorder of haematopoieitc stem cells of the myeloid lineage. It is a quantitative disorder
52
what are four disorders encompassed in MPN
- CML - Polycythemia Rubra Vera - Primary Myelofibrosis - Essential Thrombocytopenia
53
what mutation is associated with essential thrombocytopenia, primary myelofibrosis and polycythemia ruby vera
JAK2
54
what is primary myelofibrosis
MPN leading to bone marrow fibrosis, extra medullary haematopoiesis and massive splenomegaly
55
what age does primary myelofibrosis occur
Incidence increases with age
56
what mutation is associated with primary myelofibrosis
JAK2
57
how does primary myelofibrosis present clinically
- Massive splenomegaly - Lethargy - Weight-Loss
58
what is polycythaemia rubra vera
Condition of increased RBC
59
what must polycythaemia rubra vera be distinguished from
- Relative polycythaemia | - Secondary polycythaemia
60
what is relative polycythaemia
Apparent increase in RBC, due to plasma deficiency
61
what is secondary polycythaemia
Increase in RBC secondary to chronic hypoxia (eg. COPD)
62
what mutation is associated with polycythaemia rubra vera
JAK2
63
what is the triad of polycythaemia rubra vera
- Neurological changes - Visual changes - Mucosal bleeding
64
what dermatological skin is associated with polycythaemia rubra vera
Plethora facies
65
what is the classic feature of polycythaemia vera
Pruritus following a hot bath
66
what is required to diagnose polycythaemia vera
- Raised RBC - Low EPO - Hypercellular bone marrow - JAK2
67
what are patients with polycythaemia vera at risk of
- Venous thrombosis | - Arterial thrombosis
68
how is polycythaemia vera managed
Venesection
69
what is ruxolitinib
JAK2 inhibitor
70
what are risks of polycythaemia vera
- Venous thrombosis: VTE, Mesenteric, Renal vein thrombosis | - Arterial thrombosis: stroke, MI
71
what malignant disorder can polycythaemia vera progress to
AML
72
what is essential thrombocytheaemia
Increase in platelets
73
what mutation is associated with essential thrombocythaemia
JAK2
74
how may patients with essential thrombocytaemia present
- Burning in hands and feet - Recurrent gout - Bleeding gums - Foetal loss
75
why do patients with ET get gum bleeding
many of the platelets formed are dysfunctional
76
how is essential thrombocythaemia managed
hydroxycarbamide

Haematology (19 decks)

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