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Immunology/Haematology > Red Cells > Flashcards

Red Cells Flashcards

1
Q

What is anaemia?

A

Reduction in red cells or their haemoglobin

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2
Q

What are some aetiologies of anaemia?

A

Blood loss
Increased destruction
Lack of production
Defective production

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3
Q

Do erythrocytes have nuclei?

A

No

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4
Q

What are the most important vitamins for red blood cell formation?

A

B12

Folic acid

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5
Q

What happens when red cells get old?

A

Macrophages from spleen, gut, lymph etc. recognise old red cells and eliminate them

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6
Q

How are red cells broken down?

A

Globin amino acids
Iron from haem reutilised
Some haem broken into bilirubin

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7
Q

What form will circulating bilirubin be in when it results from liver problems?

A

Conjugated

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8
Q

What from will circulating bilirubin be in when it results from red blood cell breakdown?

A

Unconjugated

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9
Q

Where are genetic defects causing congenital anaemias?

A

Red cell membrane
Metabolic pathways
In haemoglobin

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10
Q

What is the clinical presentation of hereditary spherocytosis?

A

Anaemia
Jaundice (Unconjugated circulating bilirubin)
Splenomegaly
Pigment gallstones

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11
Q

How is hereditary spherocytosis treated?

A

Folic acid (increased requirements)
Transfusion
Splenectomy if very severe anaemia

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12
Q

What are the 2 main roles of enzymes for metabolic pathways in red cells?

A

Glycolysis

Pentose phosphate shunt

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13
Q

Where are the problems usually in red cell enzyme issues?

A 
G6P dehydrogenase (G6PD)
Pyruvate kinase
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14
Q

What is G6PD?

A

Glucose 6 phosphate dehydrogenase

Protects red cell proteins from oxidative damage

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15
Q

Why are males mostly affected by G6DP deficiency?

A

X linked
Men only have one X chromosome which is mutated
Females have another X chromosome with a normal gene so less likely to be affected

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16
Q

How does G6PD deficiency present?

A 
Anaemia
Neonatal jaundice (from infection or drugs)
Splenomegaly
Pigment gallstones
Haemolysis
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17
Q

What triggers haemolysis in G6DP deficiency?

A 
Infection
Acute illness
Fava beans (broad beans)
Antimalarials
Aspirin
Other drugs
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18
Q

What does pyruvate kinase deficiency do?

A

Reduced ATP
Increased 2,3-DPG
Cells rigid

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19
Q

What is reduced or absent globin chain production called?

A

Thalassaemia

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20
Q

What are some mutations leading to structurally abnormal globin chains?

A 
HbS (Sickle cell) disease
HbC
HbD
HbE
HbO Arab
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21
Q

What is Sickle cell disease?

A 
Normal alpha chain
Point mutations  in 2 beta chains where
Glu replaced by Val in beta chains
Deoxygenated
Haemolysed
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22
Q

What does sickle cell disease do?

A 
Endothelial activation
Promotion of inflammation
Coagulation activation
Dysregulation of vasomotor tone by vasodilator mediators (NO)
Vasal occlusion
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23
Q

How might sickle cell disease present in multiple systems?

A

Painful vaso-occlusive crises (bone)
Chest crisis when lungs affected
Stroke
Gallstones due to haemolysis

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24
Q

Does sickle cell increase infection risk?

A

Yes

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25
Q

Why is folic acid used to treat anaemias?

A

Increased demand due to red cell breakdown

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26
Q

How might severe anaemia affect babies?

A 
Present at 3-6 months of age
Expansion of ineffective bone marrow 
Bony deformities
Splenomegaly
Growth retardation
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27
Q

How are young children treated for thalassaemia?

A

Iron chelation therapy
Desferrioxamine infusions
Bone marrow transplantation

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28
Q

How can sickle cell cause vaso-occlusion?

A

Endothelial activation
Promotion of inflammation
Coagulation activation
Dysregulation of vasomotor tone by vasodilator mediators (NO)

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29
Q

What are the main components of life-long sickle cell disease prophylaxis?

A 
Vaccination
Penicillin 
Folic acid
Bone marrow transplant
Gene therapy
30
Q

How do we manage acute events of sickle cell disease?

A 
Hydration
Oxygenation
Prompt treatment of infection
Analgesia
Blood transfusion
31
Q

What is a drug used to manage sickle cell disease?

A

Hydroxycarbamide

32
Q

What is a thalassaemia?

A

Reduced or absent globin chain production

33
Q

How might severe anaemia resulting from beta thalassaemia major present?

A 
Present at 3-6 months of age
Expansion of ineffective bone marrow
Bony deformities (hair-on-end x-ray appearance)
Splenomegaly
Growth retardation
34
Q

What are the 3 main causes of inherited anaemias?

A

Red cell membrane defect
Red cell metabolism defect (enzyme problem)
Haemoglobin production defect

35
Q

What are some factors influencing normal range of Hb levels?

A 
Age
Sex
Ethnic origin
Time of day
Time to analysis
36
Q

Do men or women have higher Hb?

A

Men

37
Q

How does anaemia usually present?

A 
Tiredness
Pallor
SOB
Ankle swelling
Dizziness
Chest pain
38
Q

What might indicate malabsorption as a cause of anaemia?

A

Diarrhoea

Weight loss

39
Q

What are red cell indices?

A

Mean cell haemoglobin (MCH)
Mean cell volume (MCV)
Can give morphological description of anaemia and indicate cause

40
Q

Which test could be used to determine morphology of anaemia?

A

Blood film

41
Q

What blood film result is affected in hypochromic, microcytic anaemia?

A

Serum ferritin

42
Q

What blood film result is affected in normochromic, normocytic anaemia?

A

Reticulocyte count

43
Q

What blood film result is affect in macrocytic anaemia?

A

B12
Folate
Bone marrow

44
Q

What are the possible fates of absorbed iron?

A

Bound to mucosal ferritin and sent off
OR
Transported across basement membrane by ferroporin

45
Q

How is iron transported in plasma?

A

Bound to transferrin

46
Q

How is iron stored?

A

As ferritin

Mainly in liver

47
Q

Which part of the GI tract absorbs iron?

A

Duodenum

48
Q

What is the most common cause of anaemia?

A

Iron deficiency

49
Q

What are some clinical features of iron deficiency?

A

Hypochromic microcytic cells
Koilonychia
Atrophic tongue
Angular cheilitis

50
Q

What are the main causes of iron deficiency?

A

GI blood loss
Menorrhagia
Malabsorption

51
Q

What would increased reticulocyte count indicate?

A

Acute blood loss

Haemolysis

52
Q

What would normal/low reticulocyte count indicate?

A

Secondary anaemia
Hypoplasia
Marrow infiltration

53
Q

What are some possible causes of secondary anaemia?

A

Infection
Inflammation
Malignancy

54
Q

What is the mechanism of haemolytic anaemia?

A

Accelerated red cell destruction (retics)

55
Q

What are some congenital causes of haemolytic anaemia?

A

Hereditary spherocytosis
G6PD deficiency
Haemoglobinopathy

56
Q

What are some acquired causes of haemolytic anaemia?

A

Auto-immune
Mechanical (artificial valve)
Severe infection

57
Q

What does the direct antiglobulin test do?

A

Detect antibody or complement on red cell membrane

If positive then HA is immune mediated

58
Q

What kind of anaemia is associated with CLL or drugs?

A

Warm autoimmune haemolytic anaemia

59
Q

What kind of anaemia is associated with CHAD, infections and lymphoma?

A

Cold autoimmune haemolytic anaemia

60
Q

What kind of anaemia is associated with a transfusion reaction?

A

Alloantibody haemolytic anaemia

61
Q

What are schistocytes?

A

Red cell fragments on blood film

Indicate intravascular haemolysis

62
Q

What are some necessary blood tests if you think a patient is haemolysing?

A 
FBC
Reticulocyte count
Blood film
Serum bilirubin
LDH
Serum haptoglobin
Direct Antiglobulin Test
63
Q

How do we support marrow function in haemolytic anaemia?

A

Folic acid

64
Q

How do we treat autoimmune haemolytic anaemias?

A 
Steroids
Treat trigger (CLL, lymphoma etc.)
65
Q

What is a possible role of surgery in haemolytic anaemia?

A

Remove site of red cell destruction

E.g. splenectomy

66
Q

What kind of anaemia is indicated by B12 and folate deficiency?

A

Megaloblastic macrocytic anaemia

67
Q

What kind of anaemia is indicated by myelodysplasia, marrow infiltration and drugs?

A

Non-megaloblastic macrocytic anaemia

68
Q

How might megaloblastic anaemia present?

A

Lemon yellow tinge
Bilirubin
LDH

69
Q

What is the most common cause of B12 deficiency in Western populations?

A

Pernicious anaemia

70
Q

How is megaloblastic anaemia treated?

A

Replace B12 by IM injection

Oral folate replacement

71
Q

What is the most common enzymopathy?

A

G6PD deficiency

Immunology/Haematology (14 decks)

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