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Z Year3 Haem Conditions > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

How are myeloproliferative disorders classified?

A

cell type

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2
Q

What are different types of myeloproliferative disorders?

A
  1. RBC: polycythaemia vera
  2. WBC: Chronical myeloid leukaemia
  3. Platelets: essential thrombocythemia
  4. Fibroblasts: myelofibrosis
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3
Q

What is epidemiology of essential thrombocytopenia (ET)?

A

50-70 years

Female

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4
Q

What is myelofibrosis?

A
  • fibrosis in response to a BM malignancy

- megakaryocytic hyperplasia

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5
Q

What is epidemiology of myelofibrosis?

A

65 years and radiation

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6
Q

What symptoms and signs would you find in myelofibrosis?

A
  1. Massive hepatosplenomegaly

2. Hypermetabolic symptoms: night sweats etc

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7
Q

What tests and results would be for myelofibrosis?

A
  1. BM aspirate: ‘dry tap’ fibrosis
  2. Blood film: tear drop cells
  3. Moderate JAK2 mutation
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8
Q

What is management for myelofibrosis?

A

Marrow support + allogenic SCT

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9
Q

What is prognosis of myelofibrosis?

A

Median survival: 4-5years

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10
Q

What are symptoms and signs of ET?

A
  1. Erythromelalgia
  2. Splenomegaly
  3. Arterial and venous thrombosis
  4. Bleeding
  5. Livedo reticularis
  6. 50% asymptomatic
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11
Q

What is pathophysiology of ET?

A

Megakaryocyte proliferation in the bone marrow increases the production of platelets, causing thrombocytosis

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12
Q

What are possible DDx of ET?

A
  1. Secondary (reactive) thrombocytosis
  2. Spurious thrombocytosis
  3. Familial essential thrombocythaemia
  4. MDS
  5. Polycythaemia vera
  6. Primary myelofibrosis
  7. CML
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13
Q

What investigations and results are used in ET?

A
  1. FBC with peripheral smear: thrombocytosis + platelet count >450x10^9
  2. Iron panel: low ferritin and iron deficiency can be cause of reactive thrombocytosis
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14
Q

What is acute treatment of ET (thrombosis or bleeding)?

A

1st line: plateletpheresis

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15
Q

What is ongoing treatment for ET?

A
  1. Patient modification + obbservation
  2. cytoreductive therapy
  3. antiplatelet therapy
  4. anticoagulation
  5. interferon
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16
Q

What is treatment for ET?

A
  1. aspirin 75mg daily
  2. hydroxycarbamide if high risk
  3. alpha interferon
  4. heparin as anticoag
17
Q

What are possible complications for ET?

A
  1. AML
  2. Primary myelofibrosis
  3. Arterial and venous thrombosis
  4. Bleeding
  5. Spontaneous abortion
18
Q

What is prognosis for ET?

A

Normal life expectancy

Z Year3 Haem Conditions (18 decks)

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