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Y3 - Haem > Myeloproliferative > Flashcards

Myeloproliferative Flashcards

1
Q

What are myeloproliferative neoplasms?

A

Group of disorders characterised by clonal expansion of a stem cell population
Increased production of myeloid cells with relatively preserved maturation

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2
Q

What will a blood film be like in myeloproliferative neoplasms?

A

Polymorphic - wide variety of cells at different stages of maturation
Hypercellular

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3
Q

What is proliferation of red cells?

A

Polycythaemia vera

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4
Q

What is proliferation of white cells?

A

Chronic myeloid leukaemia

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5
Q

What is proliferation of platelets?

A

Essential thrombocythaemia

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6
Q

What is proliferation fo fibroblasts?

A

Primary myelofibrosis

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7
Q

Which myeloproliferative neoplasm is BCR-ABL1 positive?

A

CML

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8
Q

What is the Philadelphia chromosome associated with?

A

CML

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9
Q

When should you consider myeloproliferative neoplasms?

A 
Splenomegaly
Thrombosis in an unusual place
High granulocyte count (eosinophilia, basophilia, neutrophilia)
High red cell count/Hb
High platelet count
No reactive cause
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10
Q

What is the mean age of diagnosis of myeloproliferative neoplasms?

A

65 (but can be much younger)

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11
Q

What are the general features of myeloproliferative neoplasms?

A 
Can be asymptomatic
Increased cellular turnover
Splenomegaly
Marrow failure
Thrombosis
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12
Q

What are the clinical features of increased cellular turnover?

A

Gout
Fatigue
Weight loss
Sweats

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13
Q

What are the clinical features of splenomegaly?

A

Early satiety

LUQ pain

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14
Q

What are the clinical features of thrombosis in myeloproliferative neoplasms?

A

Arterial or venous (TIA, MI, abdominal vessel thrombosis, claudication)
Erythromelelgia - paint nd redness in hands and feet

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15
Q

What is polycythaemia?

A

Increase in red cells

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16
Q

What is the pathophysiology of polycythaemia vera?

A

Increased red cell mass due to increased production

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17
Q

What should polycythaemia vera be distinguished from?

A 
Secondary polycythaemia (reactive)
Pseudopolycythaemia
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18
Q

What are causes of secondary (reactive) polycythaemia?

A 
Chronic hypoxia (COPD, smoking)
Increased erythropoietin production (renal or hepatic carcinoma)
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19
Q

What is pseudo-polycythaemia?

A

Relative increase in Hb due to plasma cell depletion

20
Q

What are causes of pseudo-polycythawmia?

A

Alcohol
Obesity
Dehydration
Diuretic therapy

21
Q

What mutation causes polycythaemia vera, and how?

A

JAK2
A tyrosine kinase that controls erythropoiesis by auto-inhibition
Mutation causes loss of auto-inhibition and stimulation of red cell production in the absence of stimuli?

22
Q

How common are JAK2 mutations in polycythaemia vera?

A

Present in over 95% of patients with polycythaemia vera

23
Q

What is the presentation of polycythaemia vera?

A 
Hyperviscocity
- lethargy
- confusion
- headache
- dizziness
- visual disturbance
Splenomegaly
- abdo pain and distension
- gout
Aquagenic pruritus (itch after warm shower/bath)
Erythromelelgia
Facial plethora
24
Q

What do investigations show in polycythaemia vera?

A

JAK2 mutation positive
Bloods - high Hb, high reticulocytes, high platelets
Erythroid hyperplasia on marrow biopsy
Neutrophil alkaline phosphatase score increased

25
Q

What investigations can be done in polycythaemia to check for secondary/pseudo causes?

A

CXR
O2 saturation
Arterial blood gases
Drug history

26
Q

What is the aim of management of polycythaemia vera?

A

Reduce risk of thrombosis

27
Q

What is the management of polycythaemia vera?

A

Low risk - venesection, aspirin

High risk - hydroxycarbamide, aspirin

28
Q

What does high risk mean in polycythaemia vera?

A

> 60

Previous thrombosis

29
Q

What is essential thrombocythaemia?

A

Uncontrolled increase in platelets

30
Q

What is the pathophysiology of essential thrombocythaemia?

A

Uncontrolled proliferation of megakaryocytes causing increased production of abnormal platelets
Causes thrombosis tendance
At high levels can cause bleeding due to acquired von Willebrand disease as vWF is absorbed into abnormal platelets, reducing circulating vWF levels

31
Q

What mutations can cause essential thrombocythaemia?

A

JAK2 (not as common as PV)

CALR

32
Q

What are other causes of thrombocythaemia?

A 
Bleeding
Infection
Malignancy
Trauma/surgery
Splenectomy nad hyposplenism
33
Q

What is the presentation of essential thrombocythaemia?

A

MPN common clinical features (thrombosis, marrow failure, high cellular turnover, splenomegaly)
Vaso-occlusive complications
Bleeding

34
Q

What can thrombosis commonly cause in essential thrombocythaemia?

A

Stroke, DVT/PE
Recurrent miscarriages
Digital ischaemia (gangrene fingers and toes)
Erythromelalgia (erythema and shooting pain in limbs)
Microembolisms

35
Q

What do investigations show in essential thrombocythaemia?

A

Thrombocytosis
Marrow biopsy - hypercellular, megakaryocytes
Genetics - JAK2, CALR

36
Q

What is the management of essential thrombocythaemia?

A

Lifestyle factors to reduce thrombosis risk
Aspirin
Hydroxycarbamide if high risk for thrombosis

37
Q

What is hydroxycarbamide, how does it work and what are other similar options?

A

Cytoreductive therapy to control proliferation

Other options: anagrelide, interferon alpha

38
Q

What is idiopathic myelofibrosis?

A

Megakaryocyte proliferation causing stimulation of fibroblasts

39
Q

What is the pathophysiology of idiopathic myelofibrosis?

A

Increase in megakaryocytes is associated with increased release of platelet derived growth factor, causing stimulation of fibroblasts and bone marrow fibrosis
Fibrosis causes marrow failure, compensated for by extra medullary haematopoiesis in the liver and spleen

40
Q

What are the key blood film features of idiopathic myelofibrosis?

A

Leucoerythroblastic film appearance - both myeloid and erythroid precursors in the blood
Teardrop shaped RBCs

41
Q

What is the presentation of idiopathic myelofibrosis?

A 
Non-specific
Fever, night sweats
Abdominal pain in LUQ
Symptoms of marrow failure (bleeding, infections, anaemia)
Massive splenomegaly
42
Q

What do investigations show in idiopathic myelofibrosis?

A

Bloods - low Hb, high WCC, high platelets
Bone marrow biopsy - marrow fibrosis
Genetics - JAK2, CALR, MPL

43
Q

What are the possible mutations in idiopathic myelofibrosis in order of most to least common?

A

JAK2 > CALR > MPL

44
Q

How many people with idiopathic myelofibrosis have no mutation?

A

10% are triple negative

45
Q

What is the management of idiopathic myelofibrosis?

A

Supportive care as required for marrow suppression (blood transfusion, platelets, antibiotics, allopurinol if hyperuricaemia)
Active treatment
- bone marrow transplant
- ruxolitinib (JAK2 inhibitor)

Y3 - Haem (9 decks)

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