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Y1 - MEH > When Haemopoiesis goes wrong > Flashcards

When Haemopoiesis goes wrong Flashcards

1
Q

What are Myeloproliferative neoplasms?

A

Group of diseases of Bone Marrow in which excess cells are produced (Cancers)

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2
Q

How do Myeloproliferative neoplasms arise?

A

Genetic mutations in the precursors of the myeloid lineage in the bone marrow

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3
Q

4 Major Types of Myeloproliferative neoplasms

A

-POLYCYTHAEMIA VERA (excess erthyrocytes)
-ESSENTIAL THROMBOCYTHAEMIA (Overproduce Megakaryocytes - too many platelets)
-PRIMARY MYELOFIBROSIS (Haematopoietic tissue replaced by connective tissue, generation of blood cells impaired PANCYTOPENIA OCCURS)
-CHRONIC MYELOID LEUKAEMIA (excess Granulocytes)

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4
Q

What is Polycythaemia?

A

Disease state
When volume percent of erythrocytes in blood (Haematocrit) exceeds:
-52% males
-48% females

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5
Q

What is Absolute Polycythaemia ?
How can it be caused?

A

Increase in the number of erythrocytes increases % RBC in blood

Primary - Bone marrow abnormality
Secondary - Increased erythropoietin

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6
Q

Relative Polycythaemia

A

Decrease in plasma volume causing increased % of Erythrocytes in blood

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7
Q

What Gene mutation causes (Primary) Polycythaemia Vera? (PV)

A

Mutation in JAK2 gene

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8
Q

Clinical Features associated with Polycythaemia vera basically from blood being thicker

A

Thrombosis
Haemorrhage
Headache dizziness
Splenomegaly
Gout
burning pain in hands and feet

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9
Q

Most common cause of morbidity and death associated with Polycythaemia Vera is?

A

Thrombosis

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10
Q

What is thrombosis?

A

When blood clots block arteries or veins

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11
Q

Treating Thrombosis

A

Phlebotomy (removing excess erythrocytes)
Aspirin (has anti-platelet effect)
Hydroxycarbamide (Inhibitts DNA synthesis and so replication)

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12
Q

What is secondary Polycythaemia?

A

Increase percentage of erythrocytes in blood due to increased production of ERYTHROPOIETIN

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13
Q

What are the Causes of Secondary Polycythaemia?

A

Something that leads to increased production of Erythropoietin

Chronic Hypoxia (living at high altitude, severe COPD, heavy cigarette use or cyanotic heart disease)
Renal disease
Tumours releasing Erythropoietin

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14
Q

What is Thrombocytosis?

A

Increased platelet count (above normal range)

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15
Q

Thrombocytosis is common reaction to

A

Infections
Inflammation

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16
Q

Thrombocytosis that is caused by a Myeloproliferative neoplasm is called

A

Essential Thrombocythaemia

17
Q

What is Essential Thrombocythaemia?

A

Chronic blood cancer (myeoproliferative neoplasm)
Overproduction of platelets due to overproduction of Megakaryocytes

18
Q

What causes Essential thrombocythaemia?

A

Mutations in JAK2 genes
Mutations in Thrombopoietin receptor

19
Q

Symptoms of Essential Thrombocythaemia

A

Numbness of extremities
Thrombosis (stroke/peripheral gangrene)
Hearing and Vision disturbances (microvascular complications)
Head aches
Burning pains of hands and feet (Erythromelalgia)

20
Q

Treating for Essential Thrombocythaemia

A

Low risk = Aspirin (anti-platelet properties)
High risk = Hydroxycarbamide
Anything that reduces platelet count

21
Q

What is Thrombocytopenia?

A

Lower than normal range of platelets
Opposite of Thrombocythaemia/thrombocytosis

Inherited or Aquired

22
Q

The general principles that causes Aquried thrombocytopenia?

A

Decreased platelet PRODUCTION
Increased platelet CONSUMPTION
Increased platelet DESTRUCTION

23
Q

What is Immune thrombocytopenia purpura (ITP)

A

Autoimmune disease that causes Thrombocytopenia
Antiplatelet auto antibodies bind to platelets
T cell activit against platelets and megakaryocytes

24
Q

What is Primary myelofibrosis?

A

Myeloproliferative neoplasm where proliferation of mutated Haematopoietic stem cells then cause replacement of bone marrow with scar tissue (collagen)

25
Q

Why does Primary Myelofibrosis often show enlarged liver and spleen?

A

Mutated bone marrow progenitor cells migrate to liver and spleen (extramedullary haematopoiesis)

26
Q

What causes Primary Myelofibrosis?

A

Often mutations of JAK2 genes

27
Q

What is Secondary Myelofibrosis/when does it occur?

A

When myelofibrosis has occurred as a result of Polycythaemia Vera or essential thrombocythaemia

28
Q

Symptoms of primary myelofibrosis

A

Hepatosplenomegaly
Bruising
Fatigue (anemia symptoms)
Weight loss
Fever
Portal hypertension

29
Q

Treatment for Primary myelofibrosis

A

Mainly supportive
Hydroxycarbamide
Folic acid (help prevent anaemia)
Splenectomy
ruxolitinib (inhibitor of JAK2)

30
Q

Thinking about Leukaemia Types

A

Acute or Chronic and what lineage do the abnormal malignant cells arise from

31
Q

Acute leukaemias

A

Large number of immature blast cells overwhelm ability of bone marrow to make mature blood cells

Rapid bone marrow failure

32
Q

Chronic leukaemias

A

Slow to cause symptoms
Often as a result of a differentiated cell being malignant

33
Q

What is Chronic Myeloid Leukaemia?

A

Unregulated growth of myeloid cell in bone marrow
Causes accumulation of mature Granulocytes (mainly neutrophils)

34
Q

What causes Chronic myeloid leukaemia?

A

Chromosomal translocation called PHILADELPHIA CHROMOSOME

35
Q

What occurs as a result of the Philadelphia chromosome?

A

Oncogenes gene fusion with tyrosine kinase activity

Proliferation, differentiation and inhibition of apoptosis

36
Q

What is Aplastic anaemia?

A

Damaged bone marrow and hematopoietic stem cells
Causes PANCYTOPENIA

37
Q

Aplastic meaning

A

Inability of stem cells to generate mature blood cells

38
Q

What can cause aplastic anaemia?

A

Autoimmunity
Chemicals
Drugs
Radiation

39
Q

What is a neoplasm?

A

A Cancer

Y1 - MEH (33 decks)

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