Lecture 18: Haemotology Disorders

Question 1

RBC reference range

Answer 1

4.2-5.4 microlitre (female) or 4.7-6.1 (males)

Question 2

HB reference range

Answer 2

13-18g per 100ml blood or 130-180g/L

Question 3

Erythrocytosis

Answer 3

RBC raised in the blood
-present for 2 months to be considered clinically relevant

Question 4

What 4 pathologies can erythocytosis be classified into

Answer 4

1. Relative (plasma conc)
2. Apparent (increased haematocrit)
3. Idiopathic (no clear mechanism)
4. Absolute (congenital or acquired)

Question 5

Erythrocytosis treatment

Answer 5

Removal of blood to reduce red cell mass

Question 6

Anaemia

Answer 6

Generally considered a decrease in erythrocytes/ Hb (blood’s capacity to carry oxygen)

Question 7

WHO classification of anaemia

Answer 7

Males= <130g/L
Females= <120g/L

Question 8

Signs and symptoms of anemia

Answer 8

-fever
-angina, cardiac fever
-jaundice
-hepatomegaly, splenomegaly
-pallor
-shortness of breath
-weakness
-dark urine
-spoon nails
-tachycardia
-glossitis
-tiredness, fatigue

Question 9

What’s haemorrhagic anaemia

Answer 9

Significant loss of RBC, leading to decreased haemoglobin

ACUTE = stab wound
CHRONIC = untreated ulcer

Question 10

Why might decreased erythrocyte production occur

Answer 10

Result of bone marrow failure or reduced availability of raw materials required

Question 11

Why might erythrocytes be inefficient at their role

Answer 11

• poor diet
• autoimmunity in pernicious anaemia (lack of B12)
• renal anaemia (lack of erythropoietin)
• aplastic anaemia (cancerous destruction of bone marrow)
• drug treatment (e.g. chemo)

Question 12

Microcyte

Answer 12

Small, pale, Hb, poor erythrocytes

Question 13

Haemolytic

Answer 13

Haemolytic anaemia (rupture RBC)
- abnormal lysis of erythrocytes
- occur at any stage of cell production
- erythrocyte production may be increased to compensate
- destruction may cause haemoglobin and bilirubin accumulation

Question 14

Macrocyte

Answer 14

Very few, large, Hb rich erythrocytes

Question 15

Causes of increased erythrocyte destruction

Answer 15

• infection
• allo and auto immune response
• haemolytic uraemia syndrome
• prolonged mechanical trauma

Question 16

Treatment for haemorrhagic anaemia

Answer 16

Stop the haemorrhage, normal erythropoiesis should then restore blood

Question 17

Treatment for disorders affecting erythrocyte production

Answer 17

Need treatment at the source e.g. change of drug treatment, iron supplementation, fortification of food production

Question 18

Normal haematopoiesis

Answer 18

Maintains a balance between the number of cells in circulation, those produced and those destroyed, including the process of self-renewal

Question 19

What can cause changes to the equilibrium of haematopoiesis

Answer 19

Oncogenic genetic mutations

Question 20

Oncogenic generic mutations can become dysregulated, leading to failures in this balance including:

Answer 20

-production of inefficient cells
-accumulation of immature cells
-increased proliferation of cells
-failure of apoptosis

These give rise to a number of haematological disorders including leukaemia, lymphoma, multiple myeloma and myelodysplastic syndrome

Question 21

What is leukaemia

Answer 21

Presence of malignant haematopoiesis cells in circulating blood or bone marrow

Question 22

Lineage of malignant cells

Answer 22

Lymphoid or myeloid

Question 23

Acute leukaemia

Answer 23

• fast growing
• accumulation of ‘blast’ cells
• > 20% cells have maturational arrest
• quick development
• mostly children

Question 24

Chronic leukaemia

Answer 24

• develop slowly
• not exclusively blast cells
• cells fail to undergo apoptosis leading to accumulation
• cells aren’t efficient at their function
• older people (40+)

Question 25

Leukaemia symptoms

Answer 25

- pallor - fever - breathlessness - sweating - hepatomegaly, splenomegaly - frequent infections - bone and joint pain - ‘petechiae’ or ‘purpara’ - weight loss - palpitations - unusual bleeding - fatigue

Question 26

Induction and consolidation phase

Answer 26

Induction- destroy and remove leukaemia cells in blood and bone marrow Consolidation- prevent relapse

Question 27

Other leukaemia treatments

Answer 27

- chemotherapy - radiotherapy - bone marrow/stem cell transplants

Question 28

Thrombosis

Answer 28

Increased clotting tendency

Question 29

Fibrinolysis

Answer 29

Increased bleeding tendency

Question 30

When do bleeding disorders occur

Answer 30

Deficiency of haemostatic components

Question 31

Haemophilia

Answer 31

Clotting disorder due to deficiency of specific components

Question 32

Haemophilia A

Answer 32

Deficiency of factor VIII -most common - may occur as spontaneous mutation to FVIII gene (30% of cases) - childhood - impairs body’s ability to make efficient blood clots

Question 33

Haemophilia B

Answer 33

Deficiency of factor IX (Christmas factor)

Question 34

Haemophilia symptoms

Answer 34

- intracranial haemorrhage - joint damage - epistaxis - haemoptysis - soft tissue bleeds and bruises - ‘Petechiae’ or ‘purpura’ - bleeding into bones and joints - haematuria - menorrhagia - prolonged bleeding after trauma/surgery - gingival bleed -

Question 35

Haemophilia treatment for prevention

Answer 35

A= octocog Alfa injection B= Nona cog Alfa injection

Question 36

Haemophilia treatment at time of injury/emergency situation

Answer 36

Does oppression to stimulate FVII production

Question 37

What have scientific advances made available as haemophilia treatment

Answer 37

Recombinant proteins