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Essential Haematology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

Define the term Myeloproliferative Disorders

A

Describes a group of conditions arising from marrow stem cells and characterized by clonal proliferation of one or more haematopoietic components in the bone marrow and often liver and spleen

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2
Q

List three Myeloproliferative Disorders

A

Polycythemia rubra vera (PRV)
Essential thrombocytopenia (ET)
Myelofibrosis

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3
Q

What is the common aetiology of Polycythemia Rubra Vera (PRV), Essential thrombocytopenia (ET), Myelofibrosis

A

Acquired mutation of the cytoplasmic tyrosine kinase

JAK2 mutation

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4
Q

What is polycythemia (erythrocytosis)

A

Defined as an increase in the haemoglobin concentration above the upper limit of normal for the patient’s age and sex

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5
Q

What are the two classifications of Polycythemia

A

Absolute Polycythemia

Relative/Pseudopolycythemia

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6
Q

Describe Absolute Polycythemia

A

This is when the red cell mass (volume) is raised

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7
Q

Describe Relative/ Pseudopolycythemia

A

Red cell volume is normal but the plasma volume is reduced

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8
Q

What are two subdivisions of Absolute Polycythemia

A 
Primary Polycythemia (PRV- Polycythemia rubra vera)
Secondary Polycythemia
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9
Q

Is Polycythemia rubra vera primary or secondary

A

Primary

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10
Q

What is the cause of increased red cell volume in Polycythemia (rubra) vera (Primary Polycythemia)

A

Clonal malignancy of a marrow stem cell

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11
Q

Which somatic mutation is present in haemopoietic cells in almost 100% of patients

A

JAK2 mutation

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12
Q

What are two causes of Primary Polycythemia

A

Polycythemia (rubra) vera

Familial (congenital) Polycythemia

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13
Q

What are two main causes of Secondary Polycythemia

A

Caused by compensatory erythropoietin increase

Caused by inappropriate erythropoietin increase

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14
Q

Compensatory erythropoietin increase is a cause of Secondary Polycythemia

What factors lead to this increase

A 
High altitude 
Pulmonary disease 
Cardiovascular disease 
Increased affinity haemoglobin
Heavy cigarette smoking
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15
Q

Inappropriate erythropoietin increase is a cause of Secondary Polycythemia

What factors lead to this increase

A

Renal diseases

Tumours

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16
Q

What are four factors that cause Relative Polycythemia

A

Stress
Cigarette smoking
Plasma loss : burns
Dehydration

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17
Q

What are the criteria for diagnosis of Polycythemia (rubra) vera

(Include red cell mass, and arterial oxygen saturation)

A 
Total red cell mass
       Male: >35 mL/kg
       Female: > 32mL/kg
Arterial Oxygen saturation >92%
Splenomegaly
JAK2 mutation
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18
Q

Polycythemia rubra vera usually affects which age group

A

Older people

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19
Q

What is the sex incidence of Polycythemia (rubra) vera

A

Equal

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20
Q

Clinical features of Polycythemia are as a result of which three conditions caused by the Polycythemia

A

Hyperviscosity
Hypervolemia
Hypermetabolism

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21
Q

What are some clinical presentations of Polycythemia (Rubra) Vera

A
  • Headaches
  • Dyspnoea
  • Blurred vision
  • Night sweats
  • Pruritus (itchy skin) after hot bath
  • Plethoric appearance
  • Splenomegaly
  • Hemorrhage or Thrombosis
  • Gout
  • Peptic Ulceration
  • Hypertension
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22
Q

Splenomegaly is found in what percentage of pts with Polycythemia (Rubra) Vera

A

75%

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23
Q

Laboratory Findings//

Describe haemoglobin, hematocrit, and red cell count in Polycythemia (Rubra) Vera

A

All increased

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24
Q

Laboratory Findings//

Describe Neutrophil levels in Polycythemia (Rubra) Vera

A

Neutrophils Leucocytosis

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25
Q

Laboratory Findings//

Neutrophil Leucocytosis is seen in what fraction of patients with Polycythemia (Rubra) Vera

A

Over half

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26
Q

Laboratory Findings//

A raised platelet count is seen in what percentage of Polycythemia (Rubra) Vera pts

A

Platelet count present in about half of pts

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27
Q

Laboratory Findings//

What mutation is present in the bone marrow and peripheral blood granulocytes in nearly 100% of Polycythemia (Rubra) Vera pts

A

JAK2 mutation

28
Q

Laboratory Findings//

The JAK2 Mutation is present in which structures of pts with Polycythemia rubra vera

A

Bone marrow

Peripheral blood granulocytes

29
Q

What is the score of Neutrophil alkaline phosphatase (NAP) in Polycythemia rubra vera

A

Usually increased

30
Q

Laboratory Findings//

What is the change in the level of B12 seen in Polycythemia rubra vera

A

Increased

31
Q

Why is B12 and B12 binding capacity increased in Polycythemia rubra vera

A

Because of an increase in haptocorrin

32
Q

Laboratory Findings//

Describe serum erythropoietin in Polycythemia rubra vera

A

Low

33
Q

What happens to blood viscosity in Polycythemia rubra vera

A

Increased

34
Q

List five treatment methods for Polycythemia Rubra Vera

A 
Venesection
Cytotoxic Myelosuppression
Phosphorus-32 Therapy 
Inerferon
Aspirin
35
Q

What is the aim of Polycythemia Rubra Vera Treatment

A

To maintain a normal blood count

Haematocrit -0.45
Platelet count below - 400 x 10^9/L

36
Q

Does venesection Control platelet count

A

No

37
Q

When is Cytotoxic Myelosuppression Treatment for Polycythemia Rubra Vera considered ?

A

If there is poor tolerance of venesection

38
Q

List two Cytotoxic drugs used In Myelosuppression of Polycythemia Rubra Vera

A

Hydroxycarbamide (hydroxyurea)

Busulfan

39
Q

List three side effects of Hydroxyurea

A

Nausea
Skin toxicity
Myelosuppression

40
Q

Which group of patients with Polycythemia Rubra Vera is given Phosphorus-32 therapy

A

This is only used for older patients with severe disease

41
Q

Even though P-32 is the most effective myelosuppressive agent, why isn’t it popularly administered as Polycythemia Rubra Vera Treatment

A

Concern about late development of leukemia limits its use

42
Q

What is the first line of drug for patients with Polycythemia Rubra Vera less than 40yrs

A

Alpha Interferon

43
Q

How does Interferon work in treatment for Polycythemia Rubra Vera

A

Subcutaneously suppresses excess proliferation in the marrow and has produced good haematological responses

44
Q

What is the effect of Aspirin on Polycythemia Rubra Vera

A

Reduces thrombotic complications , without an increased risk of major haemorrhage

45
Q

What is the prognosis and median survival of pts with Polycythemia Rubra Vera

A

Good prognosis

Survival 10-16 yrs

46
Q

What percentage of pts with Polycythemia Rubra Vera PRV experience a transition from PRV to myelofibrosis

A

30% of pts

47
Q

What causes Primary Familial (congenital) Polycythemia

A

Mutation of von Hipp- Lindau gene which creates abnormal oxygen sensing with increased erythropoietin production

48
Q

Which test is done to confirm Polycythemia

A

PCR (polymerase chain reaction) test for the JAK2 mutation

49
Q

What is Essential Thrombocythaemia

A

In this condition there is a sustained increase in platelet count, because of megakaryocyte proliferation and overproduction of platelets

50
Q

What is the central diagnostic feature for Essential thrombocythaemia

A

A persisting platelet count of >400 x 10^9/L

Other diagnostic causes of a raised platelet count need to be fully excluded before the diagnosis can be made

51
Q

What are two types of causes of a raised platelet count

A

Reactive

Endogenous

52
Q

What are 6 Reactive causes of a raised platelet count

A 
Haemorrhage, trauma, postoperative 
Chronic iron deficiency 
Malignancy 
Chronic infections 
Connective tissue disease
Post- splenectomy
53
Q

What are four endogenous causes of a raised platelet count

A

Essential thrombocythaemia

Sometimes- Polycythemia vera, myelofibrosis, chronic myeloid leukemia

54
Q

What are the dominant clinical features of Essential thrombocythaemia

A

Thrombosis and

Haemorrhage

55
Q

What is a characteristic symptom of Essential thrombocythaemia

A

Erythromelalgia (burning sensation felt in the hands or feet and promptly relieved by aspirin)

56
Q

What percentage of pts with Essential thrombocythaemia have palpable Splenomegaly

A

40%

Others have splenic atrophy because of infarction

57
Q

Which group of pts with Thrombocythaemia is most at risk

A

Those over 60yrs , with a platelet count >1000 x 10^9/L

58
Q

What is the aim of treatment of pts at high risk with Thrombocythaemia

A

Keep the platelet count as low as 600 x 10^9/ L

59
Q

What is the most widely used treatment for Thrombocythaemia

A

Hydroxyurea

Alpha Interferon used in younger pts

60
Q

What is the course of Essential thrombocythaemia

A

Usually stationary for 10-20 yes and progresses to myelofibrosis

61
Q

What is the predominant feature of myelofibrosis

A

Progressive generalized reactive fibrosis of the bone marrow in association with the development of haemopoiesis in the spleen and liver

62
Q

List four Clinical Features of Myelofibrosis

A
  • insidious onset in older people with symptoms of anaemia
  • Symptoms resulting from massive Splenomegaly (Abdominal discomfort , pain or indigestion)
  • Hypermetabolic symptoms (such as weight loss, anorexia, fever and night sweats)
  • Bleeding problems, bone pain or gout
63
Q

What is the haemoglobin level in Myelofibrosis

A

Normal or high

64
Q

What type of blood film is seen in Myelofibrosis

A

Leucoerythroblastic blood film

Red cells show characteristic ‘tear-drop’ poikilocytes

65
Q

Which test is done to observe Myelofibrosis

A

Trephine biopsy

Essential Haematology (14 decks)

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