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MEH > When haematopoesis goes wrong > Flashcards

When haematopoesis goes wrong Flashcards

1
Q

What are myeloid proliferative neoplasms?

A

A group of diseases of the bone marrow, in which excess cells are produced.

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2
Q

Why do myeloproliferative neoplasms occur?

A

Genetic mutations in the precursors of myeloid lineage in bone marrow

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3
Q

4 main types of myeloproliferation neoplasms

A

Polycythaemia vera- excess rbcs
Essential thrombocythaemia- - excess production of megakaryocytes leading to excess platelets
Primary myeliofibrosis— leads to pancytopenia
Chronic myeloid leukaemia- excess granuloma tea

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4
Q

Polycythaemia

A

Disease where the volume percentage of erythrocytes in the blood in males is greater than 52% or 48% in females

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5
Q

What does polycythaemia arise from?

A

Myeloproliferative neoplasms in the bone marrow

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6
Q

Types of polycythaemia

A

Relative- there is a decrease in plasma volume so an increase in the relative percentage of red blood cells could be due to dehydration
Absolute- there is an increase in the number of rbcs

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7
Q

Types of absolute polycythaemia

A

Primary- originates in the bone marrow- polycythaemia bone marrow
Secondary- caused by increased levels of erythropoietin

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8
Q

Types of absolute secondary polycythaemia

A

Physiological response to hypoxia- normal

Abnormal production

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9
Q

Reasons for absolute secondary polycythaemia- physiological response

A

High altitude

Chronic lung disease

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10
Q

Reasons for absolute secondary polycythaemia- abnormal production

A

Renal carcinoma

Renal artery stenosis

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11
Q

Causes of polycythaemia vera

A

Most cases are caused by a genetic mutation of the gene coding for Janus kinase 2 (JAK2)- a tyrosine kinase which normally works in response to erythropoietin

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12
Q

Clinical features of polycythaemia vena

A 
Stickier blood
Thrombosis
Haemorrhage
Headache and dizziness
Excess body
Pruritus
Splenomegaly
Gout
Arthritis
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13
Q

Treatment of polycythaemia vena

A

Take blood to keep the haematocrit below 45%- although if the patient has poor tolerance to venesection may use hydroxycarbamide
May also use aspirin- anti platelet drug

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14
Q

Thrombocytosis

A

Increase in platelet count compared to normal range of a person of the same gender and age

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15
Q

Reasons for thrombocytosis

A

Reaction to infection and inflammation
Result from myeloproliferative neoplasm - known as essential thrombocythaemia
The splenic platelets can also be redistributed to the blood stream

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16
Q

What is essential thrombocytosis?

A

Chronic blood cancer- over production of platelets by megakaryocytes in the bone marrow.

17
Q

Common symptoms of essential thrombocytosis

A 
Numbness in extremities
Thrombosis
Disturbances in hearing and vision
Headaches
Burning sensation in the hands or feet
18
Q

Treatments for thrombocytosis

A

Low risk for bleeding- aspirin

High risk for bleeding- hydoxycarbamide

19
Q

Thrombocytopenia

A

Low level of platelets

20
Q

Reasons for thrombocytopenia

A

Inherited thrombocytopenia,
Acquired thrombocytopenia- decrease in platelet production, increased combustion of platelets or increased destruction of platelets

21
Q

Symptoms of acquired thrombocytopenia

A

Bleeding gums, nose bleeds, heavier/ long periods, bruising and petechiae,

22
Q

Immune thrombocytopenia purpura

A

An autoimmune disease- isolated thrombocytopenia- can be acute or chronic

23
Q

Reasons for decreased platelet production

A 
B12 or folate deficiency
Acute leukaemia
Aplastic anemia 
Liver failure- leading to decreased production of thrombopoetin
Sepsis
Cytotoxic chemotherapy
24
Q

Reasons for increased platelet consumption

A

Massive haemorrhage
Disseminated intravascular coagulation
Thrombotic thrombocytopenia purpura

25
Q

Reasons for increased platelet destruction

A

Autoimmune thrombocytopenia purpura
Drug induced e.g. heparin
Hypersplenism- destruction and splenic pooling of platelets

26
Q

What is autoimmune thrombocytopenia purpura?

A

Normal bone marrow and a low platelet curve which causes a purpuric rash and an increased tendency to bleed. There is T cell action against platelets and anti platelet antibodies

27
Q

What is disseminated intravascular coagulation?

A

Small clots form in body blocking small blood vessels- platelets are used up so they cannot prevent bleeding

28
Q

What is thrombotic thrombocytopenia purpura?

A

A rare blood disorder where thrombi block small blood vessels which can damage organs such as the kidney

29
Q

What is primary myelofibrosis?

A

A myeloprogenerative neoplasm where the proliferation of mutated haematopoeitic cells eventually lead to the replacement of the bone martrow with scar tissue preventing the production of blood from the bone marrow

30
Q

Why do patients with primary myelofibrosis show an enlarged liver and spleen?

A

Extracellular haematopoesis occurs meaning that the liver and spleen are needed to make blood cells

31
Q

Symptoms of myelofibrosis

A 
Hepatosplenomegaly
Bruising
Fatigue
Weight loss
Fever
Increased sweating
Portal hypertension
32
Q

What is secondary myelofibrosis?

A

Myelofibrosis as a consequence of polycythaemia vera and essential thrombocythaemia

33
Q

Treatments for myelofibrosis

A

Hydroxycarbomide
Folic acid
Allopurinol
May require transfusions and sometime splenectomy

34
Q

Prognosis for someone with myelofibrosis

A

Medain survival 5 years

Treatment is only supportive

35
Q

Chronic myeloid leukaemia

A

Unregulated growth of myeloid cells in the bone marrow leading to the accumulation of mature granulocytes as well as myelocytes

36
Q

What is aplastic anemia?

A

Caused by damage to the bone marrow and haematopoetic stem cells leading to pancytopenia

37
Q

What is pancytopenia?

A

Anemia, leukopenia and thrombocytopenia

38
Q

Causes of aplastic anemia

A 
Genetics
Autoimmunity
Drugs
Radiation
Chemicals

MEH (6 decks)

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