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Red Cells Flashcards

(71 cards)

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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
Why is folic acid used to treat anaemias?
Increased demand due to red cell breakdown
26
How might severe anaemia affect babies?
``` Present at 3-6 months of age Expansion of ineffective bone marrow Bony deformities Splenomegaly Growth retardation ```
27
How are young children treated for thalassaemia?
Iron chelation therapy Desferrioxamine infusions Bone marrow transplantation
28
How can sickle cell cause vaso-occlusion?
Endothelial activation Promotion of inflammation Coagulation activation Dysregulation of vasomotor tone by vasodilator mediators (NO)
29
What are the main components of life-long sickle cell disease prophylaxis?
``` Vaccination Penicillin Folic acid Bone marrow transplant Gene therapy ```
30
How do we manage acute events of sickle cell disease?
``` Hydration Oxygenation Prompt treatment of infection Analgesia Blood transfusion ```
31
What is a drug used to manage sickle cell disease?
Hydroxycarbamide
32
What is a thalassaemia?
Reduced or absent globin chain production
33
How might severe anaemia resulting from beta thalassaemia major present?
``` Present at 3-6 months of age Expansion of ineffective bone marrow Bony deformities (hair-on-end x-ray appearance) Splenomegaly Growth retardation ```
34
What are the 3 main causes of inherited anaemias?
Red cell membrane defect Red cell metabolism defect (enzyme problem) Haemoglobin production defect
35
What are some factors influencing normal range of Hb levels?
``` Age Sex Ethnic origin Time of day Time to analysis ```
36
Do men or women have higher Hb?
Men
37
How does anaemia usually present?
``` Tiredness Pallor SOB Ankle swelling Dizziness Chest pain ```
38
What might indicate malabsorption as a cause of anaemia?
Diarrhoea | Weight loss
39
What are red cell indices?
Mean cell haemoglobin (MCH) Mean cell volume (MCV) Can give morphological description of anaemia and indicate cause
40
Which test could be used to determine morphology of anaemia?
Blood film
41
What blood film result is affected in hypochromic, microcytic anaemia?
Serum ferritin
42
What blood film result is affected in normochromic, normocytic anaemia?
Reticulocyte count
43
What blood film result is affect in macrocytic anaemia?
B12 Folate Bone marrow
44
What are the possible fates of absorbed iron?
Bound to mucosal ferritin and sent off OR Transported across basement membrane by ferroporin
45
How is iron transported in plasma?
Bound to transferrin
46
How is iron stored?
As ferritin | Mainly in liver
47
Which part of the GI tract absorbs iron?
Duodenum
48
What is the most common cause of anaemia?
Iron deficiency
49
What are some clinical features of iron deficiency?
Hypochromic microcytic cells Koilonychia Atrophic tongue Angular cheilitis
50
What are the main causes of iron deficiency?
GI blood loss Menorrhagia Malabsorption
51
What would increased reticulocyte count indicate?
Acute blood loss | Haemolysis
52
What would normal/low reticulocyte count indicate?
Secondary anaemia Hypoplasia Marrow infiltration
53
What are some possible causes of secondary anaemia?
Infection Inflammation Malignancy
54
What is the mechanism of haemolytic anaemia?
Accelerated red cell destruction (retics)
55
What are some congenital causes of haemolytic anaemia?
Hereditary spherocytosis G6PD deficiency Haemoglobinopathy
56
What are some acquired causes of haemolytic anaemia?
Auto-immune Mechanical (artificial valve) Severe infection
57
What does the direct antiglobulin test do?
Detect antibody or complement on red cell membrane | If positive then HA is immune mediated
58
What kind of anaemia is associated with CLL or drugs?
Warm autoimmune haemolytic anaemia
59
What kind of anaemia is associated with CHAD, infections and lymphoma?
Cold autoimmune haemolytic anaemia
60
What kind of anaemia is associated with a transfusion reaction?
Alloantibody haemolytic anaemia
61
What are schistocytes?
Red cell fragments on blood film | Indicate intravascular haemolysis
62
What are some necessary blood tests if you think a patient is haemolysing?
``` FBC Reticulocyte count Blood film Serum bilirubin LDH Serum haptoglobin Direct Antiglobulin Test ```
63
How do we support marrow function in haemolytic anaemia?
Folic acid
64
How do we treat autoimmune haemolytic anaemias?
``` Steroids Treat trigger (CLL, lymphoma etc.) ```
65
What is a possible role of surgery in haemolytic anaemia?
Remove site of red cell destruction | E.g. splenectomy
66
What kind of anaemia is indicated by B12 and folate deficiency?
Megaloblastic macrocytic anaemia
67
What kind of anaemia is indicated by myelodysplasia, marrow infiltration and drugs?
Non-megaloblastic macrocytic anaemia
68
How might megaloblastic anaemia present?
Lemon yellow tinge Bilirubin LDH
69
What is the most common cause of B12 deficiency in Western populations?
Pernicious anaemia
70
How is megaloblastic anaemia treated?
Replace B12 by IM injection | Oral folate replacement
71
What is the most common enzymopathy?
G6PD deficiency

Immunology/Haematology (14 decks)

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