Chronic myeloproliferative disorders Flashcards Preview

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Flashcards in Chronic myeloproliferative disorders Deck (28)
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1

Is maturation affected in myeloproliferative disorders?

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

2

Which myeloproliferative disorder is BCR-ABL1 positive?

Chronic myeloid leukaemia

3

Which myeloproliferative disorders are BCR-ABL1 negative?

Idiopathic myelofibrosis
Polycythaemia rubra vera
Essential thrombocytopenia

(All except chronic myeloid leukaemia)

4

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

Polycythaemia rubra vera

5

Which chronic myeloproliferative disorder involves an over production of platelets?

Essential thrombocytopenia

6

Which chronic myeloproliferative disorder involves an over production of granulocytes?

Chronic myeloid leukaemia

7

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

Fatigue, weight loss and night sweats

Splenomegaly - abdominal discomfort

Thrombosis

Gout

Fibrosis

8

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

Sepsis

Marrow infiltration

9

What investigations should be done for myelofibrosis?

Blood film and FBC

Dry aspirate

Trephine biopsy

JAK2 mutation status

10

What are the treatment options for myelofibrosis?

Supportive; blood transfusion/ platelets/ antibiotics

Allogenic stem cell transplantation

Splenectomy

JAK2 inhibitors

11

What is polycythaemia rubra vera?

A chronic myeloproliferative disorder

High red blood cells

12

What are some secondary causes of polycythaemia?

COPD

Smoking

EPO secreting tumours (e.g renal or hepatocellular)

13

What are some causes of pseudopolycythaemia?

Dehydration

Diuretic therapy

Obesity

14

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

Headache

Fatigue

Itch (esp after hot baths/ showers)

Facial plethora

Burning in the fingers and the toes

Splenomegaly

Thrombosis

15

What investigations should be done for polycythaemia?

FBC and blood film

JAK2 mutation status

Investigate for other causes than rubra vera

16

What are the treatment options for polycythaemia rubra vera?

Venesection

Aspirin

Hydroxycarbamide (cytotoxic oral chemotherapy)

17

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

<0.45

18

What is essential thrombocytopenia?

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

19

What investigations should be done for essential thrombocytopenia?

Exclude CML

JAK2 mutation status

CALR (calreticulin) in those without JAK2 mutation

20

What are the treatment options for essential thrombocytopenia?

Aspirin

Hydroxycarbamide (cytoreductive therapy)

21

What is the relevance of the philadelphia chromosome in CML?

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

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

Asymptomatic

Splenomegaly

Hypermetabolic syndromes

Gout

Priapism

23

How is chronic myeloid leukaemia managed?

Tyrosine kinase inhibitors (imatinib)

Stem cell/ bone marrow transplantation

24

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

Reactive causes

25

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

Infection

Physiological post-surgery

Steroids

26

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

Infection

Malignancy

Iron deficiency

Blood loss

27

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

Pseudopolycythaemia

secondary polycythaemia

28

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

Secondary polycythaemia