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Cancer Care > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

What are myeloproliferative neoplasms?

A

Group of neoplasms involving overproduction of blood cells

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2
Q

What is polycythaemia vera?

A

Erythroid hyperplasia

Patients also get overproduction of neutrophils and platelets

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3
Q

At what age is polycythaemia vera prevalent?

A

65-74

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4
Q

What are the common signs and symptoms of Polycythaemia Vera?

A 
Thrombotic events - MI, DVT, Stroke, Budd Chiari
Headache and blurred vision 
Pruritus - after warm shower
Plethoric appearance - red faced
Splenomegaly
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5
Q

What is Budd Chiari syndrome?

A

Hepatic vein thrombosis

Lead to tender hepatomegaly, ascites, and sudden severe abdominal pain

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6
Q

What investigation results would you see in Polycythaemia Vera?

A

Raised Hb, haematocrit and red cell mass
Low erythropoietin
Raised WCC
Raised platelets (only in 50%)

JAK2 mutation

Hypercellular bone marrow

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7
Q

How is polycythaemia vera managed?

A 
control CV risk factors
Low dose aspirin --> hypercoagulable state
Venisection
Hydroxyurea
Ruxolitinib - JAK2 inhibitor
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8
Q

What is the prognosis for polycythaemia vera?

A

~ 14 years

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9
Q

What complication can arise from Polycythaemia Vera?

A

AML transformation

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10
Q

What is primary myelofibrosis?

A

Proliferation of haematopoietic stem cells in the marrow and at other sites leading to fibrosis

Marrow is replaced with scar tissue

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11
Q

At what age is primary myelofibrosis most prevalent?

A

60yo

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12
Q

What signs and symptoms are commonly seen in primary myelofibrosis?

A

Typically elderly present with anaemia
B Symptoms
Massive splenomegaly

\+/-
Spontaneous bleeding
DCIS
Oesophageal varices
Petechiae
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13
Q

What investigation findings would you expect with myelofibrosis?

A

Anaemia
WCC and platelets high in early disease then low

Bone marrow aspiration = dry tap
So need to do trephine biopsy

“Tear drop” poikilocytes (RBC’s) on blood film

High urate and LDH

Homozygous JAK2 or MPL mutation

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14
Q

How is primary myelofibrosis managed?

A

Allogenic stem cell transplant is the only cure

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15
Q

What is the palliative/symptomatic management for primary myelofibrosis?

A

Red cell transfusion/EPO for anaemia

hydroxyurea or splenectomy - splenomegaly

prednisolone - cytopenic

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16
Q

What are the complications of primary myelofibrosis?

A

progress to blast phase - like AML
portal hypertension
splenic infarct
immune deficiency

17
Q

What is essential thrombocythaemia?

A

Failure in platelet production regulation

Malignant megakaryocyte proliferation

18
Q

At what age do people get essential thrombocythaemia?

A

60yo

19
Q

What are the common signs and symptoms of essential thrombocythaemia?

A

Burning sensation in hands

Mixed thrombosis and bleeding in any system:
Dusky extremities
TIA and amaurosis fugax
Arterial and Venous thrombosis (leg, MI, hepatic, renal)
Bleeding - GI, gums, eyes, urinary

Sweating/Fever
Hepato/splenomegaly

20
Q

Why can you see bleeding in essential thrombocythaemia?

A

clotting factors run out

21
Q

How do you diagnose essential thrombocythaemia?

A

Diagnosis of exclusion

22
Q

What is found in investigations of essential thrombocythaemia?

A

Platelet count >600
High WCC, RBC but low Hb

thrombocytes on blood spear

hypercellular bone marrow with giant megakaryocytes and increased reticulin

Impaired platelet aggregation

Philadelphia chromosome absent

Heterozygous JAK2 or MPL mutation

23
Q

What factors puts a patient with essential thrombocythaemia at high risk?

A

> 60yo
History of thrombosis
Very high platelet count

24
Q

How do you manage Essential Thrombocythaemia patients?

A

Aspirin
Hydroxyurea
Interferon-alpha

25
Q

Why is hydroxyurea used in essential thrombocythaemia?

A

anti-metabolite –> reduce platelets

26
Q

What are the main complications of essential thrombocythaemia?

A

Thrombosis
Haemorrhage
AML transformation

Cancer Care (25 decks)

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