Brainscape's Knowledge GenomeTM

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


See full index

HAEMATOLOGY > Chronic myeloproliferative disorders > Flashcards

Chronic myeloproliferative disorders Flashcards

1
Q

Is maturation affected in myeloproliferative disorders?

A

There is increased production of cells but in contrast to leukaemia, maturation is relatively preserved
- so there are lots of normal cells

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

Which myeloproliferative disorder is BCR-ABL1 positive?

A

Chronic myeloid leukaemia

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

Which myeloproliferative disorders are BCR-ABL1 negative?

A

Idiopathic myelofibrosis
Polycythaemia rubra vera
Essential thrombocytopenia

(All except chronic myeloid leukaemia)

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

Which chronic myeloproliferative disorder involves an over production of red cells?

A

Polycythaemia rubra vera

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

Which chronic myeloproliferative disorder involves an over production of platelets?

A

Essential thrombocytopenia

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

Which chronic myeloproliferative disorder involves an over production of granulocytes?

A

Chronic myeloid leukaemia

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

What are some of the common signs and symptoms of myeloproliferative disorders?

A

Fatigue, weight loss and night sweats

Splenomegaly - abdominal discomfort

Thrombosis

Gout

Fibrosis

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

Blood films in myelofibrosis show tear-dropped shaped RBCs and leucoerythroblastic film. What are some other causes of this kind of film?

A

Sepsis

Marrow infiltration

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

What investigations should be done for myelofibrosis?

A

Blood film and FBC

Dry aspirate

Trephine biopsy

JAK2 mutation status

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

What are the treatment options for myelofibrosis?

A

Supportive; blood transfusion/ platelets/ antibiotics

Allogenic stem cell transplantation

Splenectomy

JAK2 inhibitors

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

What is polycythaemia rubra vera?

A

A chronic myeloproliferative disorder

High red blood cells

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

What are some secondary causes of polycythaemia?

A

COPD

Smoking

EPO secreting tumours (e.g renal or hepatocellular)

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

What are some causes of pseudopolycythaemia?

A

Dehydration

Diuretic therapy

Obesity

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

What are some of the clinical features of polycythaemia rubra vera?

A

Headache

Fatigue

Itch (esp after hot baths/ showers)

Facial plethora

Burning in the fingers and the toes

Splenomegaly

Thrombosis

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

What investigations should be done for polycythaemia?

A

FBC and blood film

JAK2 mutation status

Investigate for other causes than rubra vera

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

What are the treatment options for polycythaemia rubra vera?

A

Venesection

Aspirin

Hydroxycarbamide (cytotoxic oral chemotherapy)

17
Q

Venesection is a treatment option for polycythaemia rubra vera. What is the aim for haematocrit levels?

A

<0.45

18
Q

What is essential thrombocytopenia?

A

Uncontrolled production of abnormal platelets which predisposes to thrombosis and abnormal bleeding

19
Q

What investigations should be done for essential thrombocytopenia?

A

Exclude CML

JAK2 mutation status

CALR (calreticulin) in those without JAK2 mutation

20
Q

What are the treatment options for essential thrombocytopenia?

A

Aspirin

Hydroxycarbamide (cytoreductive therapy)

21
Q

What is the relevance of the philadelphia chromosome in CML?

A

The philadelphia chromosome results in a new gene; BCR-ABL1

This gene product is a tyrosine kinase which causes abnormal phosphorylation which causes the haematological changes seen in CML

22
Q

What are some of the clinical features of chronic myeloid leukaemia?

A

Asymptomatic

Splenomegaly

Hypermetabolic syndromes

Gout

Priapism

23
Q

How is chronic myeloid leukaemia managed?

A

Tyrosine kinase inhibitors (imatinib)

Stem cell/ bone marrow transplantation

24
Q

What is the most common cause of high cell counts; myeloproliferative disorders or reactive causes?

A

Reactive causes

25
Q

What are some examples of reactive causes which can cause a high granulocyte count?

A

Infection

Physiological post-surgery

Steroids

26
Q

What are some examples of reactive causes which can cause a high platelet count?

A

Infection

Malignancy

Iron deficiency

Blood loss

27
Q

What are some examples of reactive causes which can cause a high red cell count?

A

Pseudopolycythaemia

secondary polycythaemia

28
Q

A high Hb level in a patient without any relevant history is suggestive of which condition?

A

Secondary polycythaemia

HAEMATOLOGY (10 decks)

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