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Haematology + Immunology > Red Cells 1 > Flashcards

Red Cells 1 Flashcards

1
Q

What is anaemia?

A

Reduction in red cells or their haemoglobin content

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2
Q

Substances required for red cell production

A

Metals:IRON, copper, cobalt, manganese
Vitamins: B12, FOLIC ACID, thiamine, vit B6
Amino acids
Hormones: ERYTHROPOIETIN, androgens, thyroxine

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3
Q

Describe red cell breakdown

A

Occurs in reticuloendothelial system

After 120 days rbc broke down into:

  • Globin (amino acids reused)
  • Haem (iron recycled into haemoglobin, haem converted into bilirubin)
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4
Q

What are the main components of a mature red blood cells

A

Membrane filled with enzymes and haemoglobin

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5
Q

Examples of congenital anaemia?

A

Hereditary Spherocytosis
G6PD deficiency
Thalassemia,
Sickle Cell disease

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6
Q

What type of inheritance is hereditary spherpcytosis

A

Autosomal dominant

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7
Q

What structural proteins in red cell membrane does hereditary spherocytosis affect

A 
Ankyrin, 
Alpha spectrin, 
Beta specctrin, 
Band 3, 
Protein 4.2
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8
Q

What shape are hereditary spherocytosis red blood cells

A

Spherical

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9
Q

What is the reticuloendothelial system

A

removes immune complexes from the circulation

Includes:
monocytes of the blood,
macrophages in connective tissue, 
lymphoid organs, 
bone marrow, 
bone, 
liver, 
lung
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10
Q

Clinical presentation of hereditary spherocytosis

A

Anaemia,
Jaundice,
Splenmegaly,
Pigment gallstones

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11
Q

Treatment of hereditary spherocytosis

A

Folic acid,
Transfusion,
Splenectomy (if severe)

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12
Q

Examples of some rare genetic anaemia membrane disorders

A

Hereditary elliptocytosis,
Hereditary pyropoikilocytosis,
South East Asian ovalocytosis

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13
Q

What provides red cells with energy and protects from oxidative damage

A

Provides energy- glycolysis

Oxidative damage protection- pentose phosphate shunt (with G6PD enzyme)

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14
Q

How does Glucose 6 phosphate dehydrogenase protect red cell proteins from oxidative damage

A

Produces NADPH

Which reduces glutathione (which scavenges and detoxifies reactive oxygen species)

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15
Q

How is G6PD deficiency inherited

A

X-linked

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16
Q

Pro and Con of G6PD deficiency

A

Pro- protects against malaria

Con- cells vulnerable to oxidative damage

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17
Q

How do cells appear in G6PD deficiency

A

Blister cells

Bite cells

18
Q

Clinical presentation of G6PD deficiency

A

Variable degrees of anaemia
Neonatal jaundice
Splenomegaly
Pigment gallstones

19
Q

Triggers to haemolysis in G6PD deficiency

A

Infection,
Acute illness eg DKA
Broad (fava) beans
Drugs- antimalarials, sulphonamides and sulphones, antibacterials, analgesics (aspirin), antihelminthics (B-naphthol), miscellaneous (vitamin K analogues, probenecid, methylene blue)

20
Q

What is Pyruvate kinase deficiency

A

Reduced ATP and increased 2,3-DPG

Causes anaemia, jaundice, gallstones

21
Q

What is Haemoglobin made up of

A

2 alpha chains
2 beta chains
Iron,
Haem

Hb A (aaBB) 
Hb A2 (aadeltadelta)
Hb F (aayy)
22
Q

Function of haemoglobin function

A

Gas exchange:
-O2 to tissues,
CO2 to lungs

Oxygen dissociation curve:

  • shifts as a compensatory mechanism
  • “Bohr effects”
    • acidosis
    • hyperthermia
    • hypercapnia
23
Q

What are haemoglobinopathies

A

Inherited abnormalities of haemoglobin synthesis

24
Q

Examples of haemoglobinopathies

A

Thalassaemia

Sickle cell

25
Q

What is Thalassaemia

A

Reduced or absent globin chain production
can be alpha thalassaemia (mutation/ deletion in alpha gene)
Or beta thalassaemia (mutation/deletion in beta genes)

26
Q

What is sickle cell disease

A

Mutations leading to structurally abnormal globin chain

Sickle haemoglobin composed of haem molecule and 2alpha chains, 2 beta (sickle) chains (due to point mutations)

27
Q

What kind of inheritance is thalassemia

A

Autosomal recessive

28
Q

Consequences of HbS polymerisation

A

Haemolysis leading to vaso-occlusion

29
Q

Sickle cell disease clinical presentation

A 
Cerebral infarcts
Retinopathy 
Cardiomegaly
Congestive heart failure 
Cholelithiasis 
Renal infarcts- haematuria 
Pulmonary infarcts- pneumonia 
Splenomegaly 
Infarcts of extremities
Vaso-occlusion
Ulcer of leg 
Aseptic bone necrosis- osteomyelitis 
Bone marrow hyperplasia
30
Q

Complications of sickle cell disease

A

Painful vaso-occlusive crisis of bone
Chest crisis
Stroke
Increased infection risk due to hyposplenism
Chronic haemolytic anaemia (gallstones, aplastic crisis)
Sequestration crisis

31
Q

Treatment of acute painful crisis

A

Opiates,
Hydration,
Oxygen,
Consider antibiotics

32
Q

Management of sickle cell disease

A

Life long prophylaxis:

  • vaccination,
  • penicillin
  • folic acid

Blood transfusion (alloimmunisation)
Bone marrow transplantation
Gene therapy

33
Q

Prognosis of homozygous alpha zero thalassaemia

A

Incomparable with life

Known as hydrops fetalis and there will be no alpha chains

34
Q

How severe is beta thalassaemia major

A

No beta chains present

Dependent on transfusion

35
Q

What is non-transfusion dependent thalassaemia

A

Range of genotypes

Unlike beta thalassaemia major there is no dependence on transfusion

36
Q

What is thalassaemia minor

A

“Trait” or carrier state

Hypochromic microcytic red cell indices

37
Q

What is beta thalassaemia major

A 
Severe anaemia
Presents at 3-6 months 
Expansion of ineffective bone marrow
Bony deformities 
Splenomegaly 
Growth retardation
38
Q

What is the life expectancy of someone with beta thalassaemia major without regular transfusions

A

<10 years

39
Q

Beta thalassaemia major treatment

A

Chronic transfusion support every 4-6 weeks

However this causes iron overloading so this needs treated
- iron chelation therapy (s/c desferrioxamine or oral deferasirox)

Bone marrow transplants

40
Q

What is monitored in someone with beta thalassaemia major

A

Ferritin levels
MRI scans

To ensure normal life expectancy

Haematology + Immunology (21 decks)

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