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Y3 Haematology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

All myeloproliferative disorders effect what stage of haematopoiesis?

A

Clonal stem cells

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2
Q

Is the marrow hypercellular, normocellular or hypocellular in myeloproliferative disorders?

A

Hypercellular

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3
Q

______ is preserved in myeloproliferative disorders

A

Maturation

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4
Q

Myeloproliferative disorders are a type of reactive change. T or F

A

False - myeloproliferative disorders are never reactive

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5
Q

What are the potential complications of all myeloproliferative disorders?

A

AML

Secondary myelofibrosis

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6
Q

How are myeloproliferative disorders classified?

A

BCR:ABL +ve
BCR:ABL -ve

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7
Q

What are the causes of BCR:ABL positive myeloproliferative disorders?

A

CML

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8
Q

What are the causes of BCR:ABL negative myeloproliferative disorders?

A

Polycythaemia rubra vera
Essential thrombocytheamia
Primary myelofibrosis

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9
Q

What lineages are effected in myeloproliferative disorders?

A

Myeloid

Hence may see raised granulocytes,RBCs, PLTs or Hb

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10
Q

What is the common presentation of all myeloproliferative disorders?

A 
Asymptomatic or
Splenomegaly
Weight loss
Night sweats
Fatigue
Gout
Unusual thrombosis
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11
Q

What causes gout, fatigue, night sweats and weight loss in myeloproliferative disorders?

A

Increased cell turnover

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12
Q

What effect does polycythaemia rubra vera have on:

  • RBC production
  • Hb
  • RBC numbers
  • Total red cell mass
  • HCT
A

All raised

Can also cause increased production of another lineage

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13
Q

What are the additional symptoms of polycythaemia rubra vera as well as those common to all myeloproliferative disorders?

A

Plethora
Aquagenic pruritus (itch worse in warm water)
HA
Fatigue

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14
Q

What causes the HA and fatigue in polycythaemia rubra vera?

A

Increased blood viscosity

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15
Q

What test is diagnostic of polycythaemia rubra vera and is useful in distinguishing it from secondary polycythaemia?

A

JAK2 mutation test

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16
Q

What is the management of polycythaemia rubra vera?

A

Venesect to HCT less than 0.45

Plus aspirin

17
Q

What causes secondary polycythaemia?

A

Chronic hypoxia eg COPD

Both reactive

18
Q

What causes pseudopolycythaemia?

A

Dehydration
Diuretics
Obesity

(All reactive)

19
Q

What is the pathology of essential polycythaemia?

A

Uncontrolled proliferation of abnormal platelets

20
Q

What is the additional complication in essential thrombocytheamia compared to other myeloproliferative disorders? How does this frequently present?

A

Thrombosis

Digit ischaemia

21
Q

50% of patients with essential thrombocythaemia have what mutation? A smaller percentage have what other mutation?

A

50% JAK2 positive

Also CALR mutation

22
Q

When investigating essential thrmbocythaemia, if a patient has no linked mutations what test should you do next?

A

Marrow biopsy

23
Q

What is the main management of essential thrombocythaemia?

A

Aspirin

24
Q

How does splenomegaly present?

A

Early satiety
LUQ pain / dragging sensation
Portal HTN

25
Q

What is the cause of splenomegaly in myeloproliferative disorders?

A

Extramedullary hematopoiesis

26
Q

What is the cause of primary myelofibrosis?

A

Idiopathic

27
Q

What are the S+S in primary myelofibrosis due to hypercatabolism?

A

Itch
Night sweats
Weight loss

28
Q

What is seen on blood film in primary myelofibrosis?

A 
Teardrop RBCs (poikilocytes)
Leukoerythroblastic
29
Q

Which myeloproliferative disorder requires a biopsy to diagnose?

A

Primary myelofibrosis

30
Q

What is the management of primary myelofibrosis?

A

Transfuse RBCs / PLTs

ABx

31
Q

What is the cause of secondary myelofibrosis?

A

After years of essential thrombocythaemia or polycythaemia rubra vera

32
Q

What is the commonest cause of thrombocytopenia?

A

Reactive - not MPD

Y3 Haematology (15 decks)

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