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Oncology, haematology and palliative care > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What are myeloproliferative disorders?

A

Disorders caused by clonal proliferation of haematopoietic myeloid stem cells in the bone marrow
These cells retain the ability to differentiate into RBCs, WBCs, plts, causing an excess of one or more of these.

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2
Q

Name the myeloproliferative disorder caused by proliferating RBCs

A

Polycythaemia vera (PRV)

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3
Q

Name the myeloproliferative disorder caused by proliferating WBCs

A

Chronic myeloid leukaemia (CML)

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4
Q

Name the myeloproliferative disorder caused by proliferating platelets

A

Essential thrombocythemia

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5
Q

Name the myeloproliferative disorder caused by proliferating fibroblasts

A

Myelofibrosis

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6
Q

Name the two types of polycythaemia

A

Relative

Absolute

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7
Q

Describe relative polycythaemia

A

Decreased plasma volume, normal red cell mass

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8
Q

What causes acute relative polycythaemia

A

Dehydration

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9
Q

What causes chronic relative polycythaemia

A

Obesity
HTN
High alcohol and tobacco intake

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10
Q

What is absolute polycythaemia

A

Increased RBC mass

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11
Q

How is absolute polycythaemia classically measured

A

By dilution of autologous radioactive chromium labelled RBCs

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12
Q

What causes primary absolute polycythaemia

A

Polycythaemia vera

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13
Q

What causes secondary absolute polycythaemia

A

Hypoxia - high altitudes, CLD, CCHD, heavy smoking

Increased EPO production - renal and hepatocellular carcinoma

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14
Q

What is polycythaemia vera?

A

The malignant proliferation of a clone derived from one pluripotent stem cell.
The erythroid progenitor offspring as unusual in not needing erythropoietin to avoid apoptosis
Excess proliferation of RBC, WCC, plts leading to hyper viscosity and thrombosis

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15
Q

What mutation is associated with polycythaemia vera?

A

JAK2 (JAK 2 V617F)

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16
Q

What age is polycythaemia vera commoner in?

A

> 60

17
Q

How may someone with polycythaemia vera present

A

Asymptomatic - abnormal FBC
Vague symptoms of hyper viscosity - headaches, dizziness, tinnitus, visual disturbance, itching after a hot bath and erythromelalgia, burning sensation in fingers and toes

18
Q

What signs may someone with polycythaemia vera have?

A

Facial plethora
Splenomegaly
Gout - increased urate from RBC turnover
Features of arterial or venous thrombosis

19
Q

What investigations would you do when suspecting polycythaemia vera?

A

FBC - Increased RBC, Hb, HCT, PCV, WCC, Plts
Increased B12
Marrow - hypercellularity with erythroid hyperplasia
Cytogenetics - required to differentiate from CML
Decreased serum EPO
Raised red cell mass on chromium studies and splenomegaly in the setting of normal PaO2 is diagnostic

20
Q

Describe how you would treat polycythaemia vera

A

Aim to keep HCT <0.45 to decrease risk of thrombosis - venesection if low risk, hydroxycarbamide or alpha-interferon (if pregnant) if high risk
Aspirin 75mg daily

21
Q

Describe the prognosis of polycythaemia vera

A

Variable, many remain well for many years
Thrombosis and haemorrhage are the main complication
Transition to myelofibrosis occurs in 30% and acute leuakaemia in 5%

22
Q

What monitoring is required in polycythaemia vera?

A

FBC every 3 months

23
Q

What is essential thrombocythemia

A

A clonal proliferation of megakaryocytes leading to persistently high platelets often >1000X10^9/L with abnormal function, causing bleeding or arterial/venous thrombosis and microvascular occlusion - headache, abnormal chest pain, light-headedness and erythromelalgia

24
Q

How is essential thrombocythemia treated

A

Exclude other causes of thrombocytosis
Aspirin 75mg OD
Hydroxycarbamide for high risk patients

25
Q

List the causes of thrombocytosis

A 
May be a reactive phenomena seen with many conditions: 
Bleeding 
Infection
Chronic inflammation
Trauma
Post-surgery
Iron deficiency
Malignancy
26
Q

What is thrombocytosis

A

Platelets >450X10^9/L

27
Q

What happens in myelofibrosis

A

Hyperplasia of megakaryocytes which produce platelet derived growth factor, leading to intense marrow fibrosis and haematopoiesis in the spleen and liver

28
Q

What signs and symptoms does someone with myelofibrosis present with?

A

Massive hepatosplenomegaly

Hypermetabolic symptoms - night sweats, fever, weight loss, abdominal discomfort, bone marrow failure

29
Q

What is seen on blood film in myelofibrosis

A

Leucoerythroblastic cells (nucleated RBC) and Tear drop RBCs

30
Q

How is diagnosis of myelofibrosis made

A

Trephine

31
Q

How is myelofibrosis treated

A

Marrow support

Allogenic stem cell transplant for young people but risk of mortality

32
Q

What is the median survival for myelofibrosis

A

4-5 years

Oncology, haematology and palliative care (19 decks)

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