Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

3MB Haematology > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

Define myeloproliferative disorders

A

Uncontrolled proliferation of a single type of stem cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Myeloproliferative disorders have the potential to transform into..

A

Acute myeloid leukaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Proliferating cell line of Primary Myelofibrosis

A

Haematopoietic stem cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Proliferating cell line of Polycythaemia Vera

A

Erythroid cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Proliferating cell line of Essential Thrombocythaemia

A

Megakaryocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Low haemoglobin
High or low white cell count
High or low platelet count

A

Primary Myelofibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

High haemoglobin

A

Polycythaemia Vera

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

High platelet count

A

Essential Thrombocythaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Give examples of BCR-ABL1 positive myeloproliferative neoplasms

A

CML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Give examples of BCR-ABL1 negative myeloproliferative neoplasms

A

Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

When to consider a MPN?

A

High granulocyte count
High RBCs
High Platelet count
Eosinophilia/basophilia
Splenomegaly
Thrombosis in an unusual place

With NO reactive explanation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

MPNs are associated with mutations in certain genes..

A

JAK2
MPL
CALR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What causes Myelofibrosis?

A

In response to fibroblast growth factor released from the proliferating cells leading to pancytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

When the bone marrow is replaced with scar tissue, haematopoiesis starts to happen in other areas, known as…

A

Extramedullary haematopoiesis:

Liver, spleen, and around the spine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

A blood film in myelofibrosis can show..

A

Teardrop-shaped red blood cells
Anisocytosis (varying sizes of red blood cells)
Blasts (immature red and white cells)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

________ is a complication of polycythaemia

A

Gout

17
Q

_________ is a common complication of polycythaemia and thrombocythaemia, leading to myocardial infarction, stroke or venous thromboembolism

A

Thrombosis

18
Q

What does the clinical picture show?

A

Polycythaemia

19
Q

Ix for MPN

A

Bone marrow biopsy
Testing for the JAK2, MPL and CALR genes

20
Q

Mx for primary myelofibrosis

A

Chemotherapy (e.g., hydroxycarbamide)
Targeted therapies, such as JAK2 inhibitors (ruxolitinib)
Allogeneic stem cell transplantation

21
Q

Mx for polycythaemia vera

A

Venesection
Aspirin
Chemotherapy (typically hydroxycarbamide)

22
Q

Mx for essential thrombocythaemia

A

Aspirin
Chemotherapy (typically hydroxycarbamide)
Anagrelide

23
Q

In Myelofibrosis, the resultant release of platelet derived growth factor is thought to stimulate _________

A

Fibroblasts

24
Q

In Myelofibrosis, haematopoiesis develops in the ____ and _____

A

Liver and spleen

25
Q

Elderly person with symptoms of anaemia e.g. fatigue
Massive splenomegaly
Hypermetabolic symptoms: weight loss, night sweats etc

A

Myelofibrosis

26
Q

What does the blood film show?

A

Typical ‘tear-drop’ poikilocytes of myelofibrosis

27
Q

Anaemia
High WBC and platelet
High urate and LDH

A

Myelofibrosis

28
Q

Myelofibrosis often causes an unobtainable bone marrow. What would be an alternative?

A

Trephine biopsy

29
Q

Pruritus, typically after a hot bath
Splenomegaly
Hypertension
Hyperviscosity - arterial/
venous thrombosis
Haemorrhage
Low ESR

A

Polycythaemia vera

30
Q

Ix for PV

A

FBC
JAK2 mutation
Serum ferritin
Renal and liver function tests

31
Q

If the JAK2 mutation is negative and there is no obvious secondary cause, then test for

A

Red cell mass
Arterial oxygen sats
Abdominal US
Serum erythropoietin level
Bone marrow aspirate and trephine
Cytogenetic analysis
BFU-E culture

32
Q

Other features that may be seen in PRV include a high/low ESR and a raised/reduced leukocyte alkaline phosphatase

A

Other features that may be seen in PRV include a low ESR and a raised leukocyte alkaline phosphatase

33
Q

Diagnostic criteria for JAK2-positive polycythaemia vera

A
34
Q

Diagnostic criteria for JAK2-negative polycythaemia vera

A

Diagnosis requires A1 + A2 + A3 + either another A or two B criteria

35
Q

Causes of severe thrombocytopenia

A

ITP
DIC
TTP
haematological malignancy

36
Q

Pseudothrombocytopenia has been reported in association with the use of..

A

EDTA as an anticoagulan

3MB Haematology (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions