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Myeloproliferative Disorders Flashcards

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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
Laboratory Findings// | Neutrophil Leucocytosis is seen in what fraction of patients with Polycythemia (Rubra) Vera
Over half
26
Laboratory Findings// | A raised platelet count is seen in what percentage of Polycythemia (Rubra) Vera pts
Platelet count present in about half of pts
27
Laboratory Findings// What mutation is present in the bone marrow and peripheral blood granulocytes in nearly 100% of Polycythemia (Rubra) Vera pts
JAK2 mutation
28
Laboratory Findings// The JAK2 Mutation is present in which structures of pts with Polycythemia rubra vera
Bone marrow | Peripheral blood granulocytes
29
What is the score of Neutrophil alkaline phosphatase (NAP) in Polycythemia rubra vera
Usually increased
30
Laboratory Findings// What is the change in the level of B12 seen in Polycythemia rubra vera
Increased
31
Why is B12 and B12 binding capacity increased in Polycythemia rubra vera
Because of an increase in haptocorrin
32
Laboratory Findings// Describe serum erythropoietin in Polycythemia rubra vera
Low
33
What happens to blood viscosity in Polycythemia rubra vera
Increased
34
List five treatment methods for Polycythemia Rubra Vera
``` Venesection Cytotoxic Myelosuppression Phosphorus-32 Therapy Inerferon Aspirin ```
35
What is the aim of Polycythemia Rubra Vera Treatment
To maintain a normal blood count Haematocrit -0.45 Platelet count below - 400 x 10^9/L
36
Does venesection Control platelet count
No
37
When is Cytotoxic Myelosuppression Treatment for Polycythemia Rubra Vera considered ?
If there is poor tolerance of venesection
38
List two Cytotoxic drugs used In Myelosuppression of Polycythemia Rubra Vera
Hydroxycarbamide (hydroxyurea) | Busulfan
39
List three side effects of Hydroxyurea
Nausea Skin toxicity Myelosuppression
40
Which group of patients with Polycythemia Rubra Vera is given Phosphorus-32 therapy
This is only used for older patients with severe disease
41
Even though P-32 is the most effective myelosuppressive agent, why isn’t it popularly administered as Polycythemia Rubra Vera Treatment
Concern about late development of leukemia limits its use
42
What is the first line of drug for patients with Polycythemia Rubra Vera less than 40yrs
Alpha Interferon
43
How does Interferon work in treatment for Polycythemia Rubra Vera
Subcutaneously suppresses excess proliferation in the marrow and has produced good haematological responses
44
What is the effect of Aspirin on Polycythemia Rubra Vera
Reduces thrombotic complications , without an increased risk of major haemorrhage
45
What is the prognosis and median survival of pts with Polycythemia Rubra Vera
Good prognosis | Survival 10-16 yrs
46
What percentage of pts with Polycythemia Rubra Vera PRV experience a transition from PRV to myelofibrosis
30% of pts
47
What causes Primary Familial (congenital) Polycythemia
Mutation of von Hipp- Lindau gene which creates abnormal oxygen sensing with increased erythropoietin production
48
Which test is done to confirm Polycythemia
PCR (polymerase chain reaction) test for the JAK2 mutation
49
What is Essential Thrombocythaemia
In this condition there is a sustained increase in platelet count, because of megakaryocyte proliferation and overproduction of platelets
50
What is the central diagnostic feature for Essential thrombocythaemia
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
What are two types of causes of a raised platelet count
Reactive | Endogenous
52
What are 6 Reactive causes of a raised platelet count
``` Haemorrhage, trauma, postoperative Chronic iron deficiency Malignancy Chronic infections Connective tissue disease Post- splenectomy ```
53
What are four endogenous causes of a raised platelet count
Essential thrombocythaemia | Sometimes- Polycythemia vera, myelofibrosis, chronic myeloid leukemia
54
What are the dominant clinical features of Essential thrombocythaemia
Thrombosis and | Haemorrhage
55
What is a characteristic symptom of Essential thrombocythaemia
Erythromelalgia (burning sensation felt in the hands or feet and promptly relieved by aspirin)
56
What percentage of pts with Essential thrombocythaemia have palpable Splenomegaly
40% | Others have splenic atrophy because of infarction
57
Which group of pts with Thrombocythaemia is most at risk
Those over 60yrs , with a platelet count >1000 x 10^9/L
58
What is the aim of treatment of pts at high risk with Thrombocythaemia
Keep the platelet count as low as 600 x 10^9/ L
59
What is the most widely used treatment for Thrombocythaemia
Hydroxyurea Alpha Interferon used in younger pts
60
What is the course of Essential thrombocythaemia
Usually stationary for 10-20 yes and progresses to myelofibrosis
61
What is the predominant feature of myelofibrosis
Progressive generalized reactive fibrosis of the bone marrow in association with the development of haemopoiesis in the spleen and liver
62
List four Clinical Features of Myelofibrosis
- 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
What is the haemoglobin level in Myelofibrosis
Normal or high
64
What type of blood film is seen in Myelofibrosis
Leucoerythroblastic blood film Red cells show characteristic ‘tear-drop’ poikilocytes
65
Which test is done to observe Myelofibrosis
Trephine biopsy

Essential Haematology (14 decks)

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