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Systems- Haematology > Myleoproliferative neoplasms (MPN): PV, ET & MF > Flashcards

Myleoproliferative neoplasms (MPN): PV, ET & MF Flashcards

1
Q

What does neoplasm mean?

A

New and abnormal growth of a tissue, usually due to cancer

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2
Q

What are myeloproliferative neoplasms?

A

Blood cancers where there is an increased production of RBCs, WCs or platelets

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3
Q

What are the 3 main types of myeloproliferative neoplasms?

A

Polycythemia Vera (PV)

Essential thrombocytopenia (ET)

Myelofirbosis

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4
Q

How may polycthemia vera present?

A

Headaches

Itching

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5
Q

How may Essential thrombocythemia present?

A

Finger & toe ischemia

Gout

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6
Q

What are some of the treatments for myeloproliferative neoplasms?

A

Aspirin

Steroids

Venesection

Hydroxycarbamide

JAK2 inhibitors

Allogenic stem cell transplants

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7
Q

What is one of the main investigations used for myeloproliferative neoplasms?

A

Gene mutatuion tests

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8
Q

What 3 genes are looked for on gene mutation tests?

A

JAK2

MLB

CALR

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9
Q

What are some of the clinical featuers of myelodysplasia?

A

Splenomegaly

Weight loss

Fatigue (very very tierd)

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10
Q

What is a classic sign of myelodysplasia on a blood film?

A

Tear-drop RBCs

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11
Q

Levels of what is increased in Polychthemia vera?

A

Haematocrit

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12
Q

What type of myeloproliferative neoplasm can be casued by CO exposure and erythropoietein abuse?

A

Polycythemia vera

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13
Q

How can polycythemia vera be treated?

A

Low-dose aspirin

Stem cell transplant

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14
Q

What mutation occurs in polycythemia vera?

A

Jak-2

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15
Q

If someone presents with claudication and thrombocytopenia, what is the likely cause?

A

Polycythmic vera

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16
Q

What is another name for EP?

A

Primary erythrocytosis

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17
Q

What does ET stand for?

A

Essetnial thromboCYTHEMIA

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18
Q

What does erythrocytosis mean?

A

Increased production of RBCs

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19
Q

What happens to blood due to PV?

A

It becomes thicker -> hyperviscoity

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20
Q

Due to the excess number of RBCs in PV, what are patient’s at an increased risk of?

A

ATE and VTE

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21
Q

What type of leukemia do MPNs often progress to?

A

AML

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22
Q

What are the clincial features of PV?

A

Headaches

Blurred vision

Plethora

Epistaxis

Ithcy skin

Gout

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23
Q

How can gout occur in PV and ET?

A

As uric acid levels are increased

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24
Q

Why can PV cause headaches, blurred vision and epistaxis?

A

As hyperviscoicity occurs due to the large number of RBCs

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25
Q

What are the 2 types of PV and what is the difference between the two regarding what casues them?

A

Primary -> BM mutations

Secondary -> underlying conditons

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26
Q

What are the causes of PV?

A

JAK2 mutations

MPL mutations

CALR mutations

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27
Q

What are some of the causes of secondary erythocytosis?

A

Hypoxia

COPD

Smoking

Hypovolemia

Diuretics

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28
Q

What investigations should you do if someone presents with an increased RBC?

A

O2 levels

Bloods

EPO levels

Cytogenetics -> JAK2 mutations

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29
Q

What is the most common mutation that causes MPNs?

A

JAK2

30
Q

How is PV treated?

A

Venesection (removal of blood)

Hydroxycarbamide

Interferon

Dalteparin

31
Q

Why is venesection a treatment for PV?

A

As it just removes blood, which PV folk essentially have too much of

32
Q

Why is dalteparin given to PV patients?

A

DVT prophlyaxis

33
Q

What is the usual dosage and ROM of dalteparin?

A

5000 units

Sub-cut

34
Q

Before kicking off treating a patient with hydroxycarbamide, what must you tell them that they’re at an increased risk of?

A

Skin cancer

35
Q

What questiosn should you always ask a patient who presents with increased RBC count?

A

Do they have SOB

Do they smoke

Do they have COPD

36
Q

What is the most common cause of increased RBC count (erythrocytosis)?

A

Hypoxemia

37
Q

What are some of the causes of erythrocytosis (increased RBC count)?

A

COPD

Smoking

High altitudes

Firboids

Renal ca

PV (obvs lol)

38
Q

Why can JAK2 mutaitions cause PV and ET?

A

JAK2 gene is present on RBCs, and is activated by EPO or thrombopoeitin to stimulate the release of RBCs or plateltes.

When mutated, it doesn’t need EPO or thrombopoeitein to actiavte it, so it constanlty remains ‘switched on’ and makes a tonne of RBCs or platelets

Cheeeers eh

39
Q

What are the classic blood test results for PV?

A

Increased RBCs (mad)

Increased HB

Increased haematocrit

40
Q

What are the classic blood test results for ET?

A

Increased platlelts

41
Q

What are the classic blood test results for MF?

A

Decreased RBCs (erythocytopenia)

Decreased platlelets (thrombocytopenia)

Decreased Hb (anaemia)

Decreased WCC (lymphocytopenia)

42
Q

JAK2, MLP and CALR are alll types of mutations. What type of mutations are they?

A

Acquired

43
Q

What does MF stand for?

A

Myelofirbosis

44
Q

What is MF?

A

The scarring of bone marrow due to all the mad mutations that occur

45
Q

What are the 2 main features of MF?

A

Splenomegaly

Leucko-erythro-blast-osis

46
Q

What are some of the clinical features of MF?

A

Splenomegaly

Hepatomegaly (sometimes)

Bone & joint pain

Hearning loss

Pancytopenia

Thrombocytopenia

Lymphocytopenia

Anaemia

47
Q

What type of hypertension is MF associated with, and what are the common features that occur?

A

Portal hypertension

Common features

Splenomegaly

Osesophageal variecs

Haemorrhoids

48
Q

What are the risk factors for MF?

A

Old age (>65yo)

Environmental exposure (benzens and toulene)

Mutations

49
Q

What environmental exposures increases your risk of getting MF?

A

Benzene

Toulene

50
Q

What can MF progress to?

A

AML

51
Q

Can gout be caused by MF?

A

Yeah bro course it can xx

52
Q

What investigations should be done for MF?

A

Bloods

BM biopsy

BM aspirate

Cytogenetics

53
Q

What is the main drug used to treat MF?

A

Ruxolitinimb

Rux-o-li-in-ib

54
Q

Why can Ruxulitinimb be used to treat MF, and also PV and ET?

A

As it’s a JAK2 inhibitor

55
Q

Along with Ruxulitinimb, what other treatments can be used for MF?

A

Allogenic stem cell transplant

Regulat blood transfusions

56
Q

What would MF patients need regularly?

A

Blood transfusions

57
Q

What type of stem cell transplant can be used to treat MF?

A

Allogenic

(not your stem cells bro)

58
Q

Levels of what are massively increased in ET?

A

Platelets

59
Q

What may also be increased (obs not to the same extent as platelets) in ET?

A

WCC

60
Q

What is ET?

(not what it stands for, what it actually is lols)

A

A slow progressive disease of the BM that leads to excess platelet production

61
Q

What 2 conditions can ET develop into?

A

MF

AML

62
Q

Even tho theres tonnes of platelets in ET, bleeding can still occur. Why is this?

A

As VWF is used up by platelets, so the more platelets -> less VWF

63
Q

What 2 organs release thrombopoieitn?

A

Liver

Kidneys

64
Q

Can hyperviscocity also occur in ET, as it obvs does in PV?

A

Yeah it can

But it’s obvs cause of the increased platelets rather than RBCs dogggg

65
Q

What are the clinical features of ET?

A

Hyperviscovity features

Peripheral neuropathy

Gout

Hyperviscoity features

Headaches

Blurred vision

Epistaxis

Hedaches

Tinnitis

Peripheral neuropathy

66
Q

Hyperviscocity can occur in both ET and PV, what are the clinical features?

A

Headaches

Blurred vision

Epistaxis

Hedaches

Tinnitis

Peripheral neuropathy

67
Q

What is a sign of ET on a bone marrow aspirate?

A

Increased number of megakaryocytes

(Mega number of Megas)

68
Q

Why does ET cause an increased number of megakaryoctes in bone marrow?

A

As megakaryoctes produce platelets

And cause the mutations fire out plateletes like theres no tomorrow, then some undifferentiated platelets- which are megas- get produced too

69
Q

What is given as prophylaxis for ATE and VTE in both PV and ET?

A

75mg Aspirin

70
Q

What determines the management of ET?

A

Risk of getting an ATE or VTE

71
Q

What is the management for ET if there risk of ATE/VTE is:

A) Low

B) High

A

A- Low risk

Aspirin (75mg)

Monitor platlelt counts

B- High risk

Hydroxyurea

Inteferon

Anagrelide

Platetphresis

72
Q

Hydroyurea and anagrelide are both used to treat what MPN condition, and how do they work?

A

ET

They decrease platelet count

Systems- Haematology (26 decks)

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