Myeloproliferative Neoplasms

Question 1

Define myeloproliferative disorders

Answer 1

Uncontrolled proliferation of a single type of stem cell

Question 2

Myeloproliferative disorders have the potential to transform into..

Answer 2

Acute myeloid leukaemia

Question 3

Proliferating cell line of Primary Myelofibrosis

Answer 3

Haematopoietic stem cells

Question 4

Proliferating cell line of Polycythaemia Vera

Answer 4

Erythroid cells

Question 5

Proliferating cell line of Essential Thrombocythaemia

Answer 5

Megakaryocyte

Question 6

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

Answer 6

Primary Myelofibrosis

Question 7

High haemoglobin

Answer 7

Polycythaemia Vera

Question 8

High platelet count

Answer 8

Essential Thrombocythaemia

Question 9

Give examples of BCR-ABL1 positive myeloproliferative neoplasms

Answer 9

CML

Question 10

Give examples of BCR-ABL1 negative myeloproliferative neoplasms

Answer 10

Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia

Question 11

When to consider a MPN?

Answer 11

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

With NO reactive explanation

Question 12

MPNs are associated with mutations in certain genes..

Answer 12

JAK2
MPL
CALR

Question 13

What causes Myelofibrosis?

Answer 13

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

Question 14

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

Answer 14

Extramedullary haematopoiesis:

Liver, spleen, and around the spine

Question 15

A blood film in myelofibrosis can show..

Answer 15

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

Question 16

________ is a complication of polycythaemia

Answer 16

Gout

Question 17

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

Answer 17

Thrombosis

Question 18

What does the clinical picture show?

Answer 18

Polycythaemia

Question 19

Ix for MPN

Answer 19

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

Question 20

Mx for primary myelofibrosis

Answer 20

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

Question 21

Mx for polycythaemia vera

Answer 21

Venesection
Aspirin
Chemotherapy (typically hydroxycarbamide)

Question 22

Mx for essential thrombocythaemia

Answer 22

Aspirin
Chemotherapy (typically hydroxycarbamide)
Anagrelide

Question 23

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

Answer 23

Fibroblasts

Question 24

In Myelofibrosis, haematopoiesis develops in the ____ and _____

Answer 24

Liver and spleen

Question 25

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

Answer 25

Myelofibrosis

Question 26

What does the blood film show?

Answer 26

Typical 'tear-drop' poikilocytes of myelofibrosis

Question 27

Anaemia High WBC and platelet High urate and LDH

Answer 27

Myelofibrosis

Question 28

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

Answer 28

Trephine biopsy

Question 29

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

Answer 29

Polycythaemia vera

Question 30

Ix for PV

Answer 30

FBC JAK2 mutation Serum ferritin Renal and liver function tests

Question 31

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

Answer 31

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

Question 32

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

Answer 32

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

Question 33

Diagnostic criteria for JAK2-positive polycythaemia vera

Answer 33

Question 34

Diagnostic criteria for JAK2-negative polycythaemia vera

Answer 34

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

Question 35

Causes of severe thrombocytopenia

Answer 35

ITP DIC TTP haematological malignancy

Question 36

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

Answer 36

EDTA as an anticoagulan