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CP - Haemostatasis > Chronic Myeloproliferative Disorders and CML > Flashcards

Chronic Myeloproliferative Disorders and CML Flashcards

1
Q

What are myeloproliferative disorders?

A

Stem cell disorders of the bone marrow which are MALIGNANT

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2
Q

What is the transformation % into acute leukaemia?

A

~10%

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3
Q

What are stem cells?

A

Immature cells, initially pluripotent

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4
Q

What is PV?

A

Polycythaemia Vera - hypercellular, lots of RBC

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5
Q

What is ET?

A

Essential thrombosis - increased megakaryocytic > high platelet count (>400)

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6
Q

What is Idiopathic myelofibrosis?

A

Fibres bands laid down inside bone marrow = elongated cells, low blood count

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7
Q

What are the main points about PV

A

Affects all ages
Symptoms: thrombosis/stroke/MI as RBC = make blood thick, itching, plethoric face, headache, muzziness, malaise, tinnitus, peptic ulcer, gout

Signs: plethora, engorged retinal veins, splenomegaly

Diagnosis: persistent Hb/hct > 0.5

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8
Q

What is relative PV?

A

If dehydrated

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9
Q

What is primary PV?

A

Bone marrow condition = clone of red cells

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10
Q

What is secondary PV?

A

Hypoxia = increased production of RBC

Can increase RBC by testosterone/EPO injections

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11
Q

What are the first line tests for PV?

A

FBC, ferritin, EPO level, UE/LFT

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12
Q

What is erythropoietin?

A

A hormone produced by the kidneys

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13
Q

What is indicated by elevated EPO?

A

Secondary PV

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14
Q

What kind of cells can also produce EPO?

A

Cancer cells

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15
Q

What is a JAX2 mutation?

A

Janus kinase - signalling pathway for cytokine receptors
Controls cell proliferation
Mutation = cell cycle of EPO always on
An acquired genetic mutation

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16
Q

What % of px with PV have a JAX2 mutation?

A

~95%

17
Q

What is the treatment for PV?

A

Venesections aiming for HCT <0.45

Aspirin 75mg orally

18
Q

What is the prognosis for PV?

A

Good - 15 year median survival but risk of developing AML/myelofibrosis

19
Q

What are the causes of Reactive Thromocytosis?

A

Surgery, infection, inflammation, malignancy, IRON DEFICIENCY, hypersplenism haemolysis, drug induced > steroids, adrenaline, TPO mimetic), post-chemo rebound

20
Q

What investigations would be done for ET?

A

Platelet count - persistence platelets >450x10^9/L

21
Q

What are the first line investigations for ET?

A

FBC, blood film, ferritin, CRP, CXR, ESR

22
Q

What are the second line investigations for ET?

A

JAX2 (50%), CALR - affects reticulum in (45%), 90% of JAX2 -ve ET, bm biopsy

23
Q

What is the treatment for ET?

A

Assess thrombotic risk (age, hypertension, DM, platelet >1500)

Antiplatelet treatment > aspirin 75mg daily

Cytoreduction with HYDROXYCARAMIDE, interferon, anagredlide, P32 in high risk px with 1 or more RF

24
Q

What does hydrocarbamide do?

A

Anti platelet drug
Suppresses bone marrow
For px that can’t tolerate venesections

CP - Haemostatasis (5 decks)

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