Brainscape's Knowledge GenomeTM

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


See full index

Y3 HAEMATOLOGY > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

What are myeloproliferative disorders (MPD)?

A

Haemopoietic stem cell disorders involving increased production with preservation of maturation

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

How are myeloproliferative disorders (MPD) different from acute leukaemia?

A

Maturation is relatively preserved

Mature cells seen on film, whereas in leukaemia they are monomorphic nucleated cells (‘blasts)

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

What are the 2 groups/sub-types of myeloproliferative disorders?

A

BCR-ABL1 negative

BCR-ABL2 positive

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

Which MPD is BCR-ABL2 positive?

A

Chronic myeloid leukaemia

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

Which MPDs are BCR-ABL2 negative?

A

Essential thrombocythaemia
Polycythaemia rubra vera
Idiopathic myelofibrosis

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

When should an MPD be considered?

A

High granulocyte count (chronic myeloid leukaemia)
High red cell count (polycythemia rubra vera)
High platelet count (thrombocythaemia)
Eosinophilia/basophilia
Splenomegaly
Thrombosis in an unusual place

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

What happens in chronic myeloid leukaemia?

A

Excess proliferation of myeloid cells (granulocytes, platelets)
3-5 years chronic phase followed by “blast” crisis akin to acute leukaemia which can be fatal

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

What would the blood film of someone with chronic phase CML look like?

A

Lots of mature neutrophils

Increased primitive cells

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

What would the blood film of someone with accelerated phase CML look like?

A

Some neutrophils

Increased primitive cells

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

What would the blood film of someone with blast crisis in CML look like?

A

Monomorphic nucleated blasts (myelocytes)

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

List some clinical features of chronic myeloid leukaemia

A 
Asymptomatic
Splenomegaly (fatigue, LUQ pain)
Hypermetabolic symptoms (sweats, fever)
Weight loss
Bone pain
Gout
Miscellaneous (priapism, visual blurring)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe the bone marrow of a patient with CML

A

Increased myelocytes and eosinophils

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

Hb may be normal in chronic myeloid leukaemia. True/False?

A

True

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

Which chromosomes are affected in chronic myeloid leukaemia? What is the genetic change and resultant protein?

A

“Philadelphia” chromosomes
9 and 22 translocation
Produces tyrosine kinase which causes abnormal phosphorylation and production of BCR-ABL1

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

List some clinical features of BCR-ABL1 negative myeloproliferative disorders

A

Asymptomatic
Increased cellular turnover (gout, fatigue, weight loss, sweats)
Signs due to splenomegaly
Marrow failure (fibrosis or leukaemic transformation)
Thrombosis (arterial or venous) e.g. TIA, MI

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

What is erythromelaegia?

A

It is characterized by intense, burning pain of affected extremities, severe redness (erythema), and is associated with myeloproliferative disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is polycythaemia rubra vera?

A

High haemoglobin/haematocrit accompanied by erythrocytosis (overproduction of red cells) but can have excessive production of other lineages

18
Q

How is polycythaemia rubra vera distinguished from secondary polycythaemia?

A

Secondary polycythaemia is caused by smoking, chronic hypoxia, erythropoietin-secreting tumours

19
Q

How is polycythaemia rubra vera distinguished from pseudo-polycythaemia?

A

Pseudo-polycythaemia is caused by dehydration, diuretic therapy, obesity
i.e. plasma volume has shrunk which makes it seem like Hb is high but it is not

20
Q

List some clinical features of polycythaemia rubra vera

A

Headache
Fatigue
Itch (aquagenic puritis)
MPD features

21
Q

What is aquagenic puritis?

A

Itch worse on exposure to warm water

22
Q

List investigations for polycythaemia

A

History (triggers of secondary polycythaemia)
Examination e.g. splenomegaly
FBC, film
Investigation for secondary/pseudo causes (CXR, O2 sats/ABG, drug history)
Bone marrow biopsy

23
Q

Splenomegaly will not be present in secondary polycythaemia. True/False?

A

True

24
Q

Which gene mutation is important to investigate as a diagnostic tool in polycythaemia?

A

JAK2 - a kinase mutation present in 95% patients

Activation of erythropoeisis in absence of ligand

25
Q

How is polycythaemia rubra vera treated?

A

Venesection until haematocrit less than 0.45
Aspirin
Cytotoxic oral chemotherapy e.g. hydroxycarbamide

26
Q

What is essential thrombocythaemia?

A

Uncontrolled production of abnormal platelets, resulting in abnormal platelet function

27
Q

What other clinical features, in addition to those common to MPD is present in patients with essential thrombocythaemia?

A

Thrombosis (abnormal platelet function)

Bleeding (unpredictable risk especially at surgery)

28
Q

What gene mutations can be present in essential thrombocythaemia?

A

JAK2
CALR
MPL

29
Q

What is the characteristic bone marrow appearance of a patient with essential thrombocythaemia?

A

Lots of big platelets

Clustering of megakaryocytes

30
Q

How is essential thrombocythaemia treated?

A

Aspirin

Cytoreductive therapy to control proliferation (hydroxycarbamide, anagrelide, IFNalpha)

31
Q

What is idiopathic myelofibrosis?

A

Proliferation of an abnormal clone of hematopoietic stem cells in the bone marrow and other sites results in fibrosis of the bone marrow

32
Q

List clinical features of myelofibrosis, other than those common to MPD

A 
Marrow failure (anaemia, bleeding, infection)
Extramedullary haematopoeisis (splenomegaly)
Hypercatabolism (weight loss, cachexia)
33
Q

Describe the appearance of a blood film in myelofibrosis

A

Leucoerythroblastic
Teardrop-shaped RBC’s in peripheral blood
(RBCs squeezing out of a fibrotic bone marrow)

34
Q

What is meant by a leucoerythroblastic film?

A

Erythroblast, red cell precursor, and myelocyte, granulocyte precursors in peripheral blood

35
Q

List causes of a leucoerythroblastic film

A

Reactive (sepsis)
Marrow infiltration
Myelofibrosis

36
Q

List diagnostic tests for myelofibrosis

A

Blood film
Dry aspirate
Fibrosis on trephine biopsy
JAK2 or CALR mutation in a proportion

37
Q

List treatment options for myelofibrosis

A

Supportive care (blood transfusion, platelets, abx)
Allogenic stem cell transplantation in young
Splenectomy
JAK2 inhibitors (improve spleen size and QoL)

38
Q

Reactive causes of high cell counts are more common than myeloproliferative disorders. True/False?

A

True

39
Q

List some reactive causes of raised granulocytes

A

Infection
Post-surgery
Steroids

40
Q

List some reactive causes of raised platelets

A 
Infection
Iron deficiency
Malignancy
Blood loss
Post-splenectomy
41
Q

List some reactive causes of raised red cells

A

Dehydration
Diuretics
Secondary polycythemia (hypoxia etc.)

Y3 HAEMATOLOGY (16 decks)

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