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Haematology > Anaemias > Flashcards

Anaemias Flashcards

1
Q

what is RBC a bag of?

A

enzymes
membranes
haemoglobin

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2
Q

whats the RBC membrane comprised of?

A

skeletal proteins that are responsible for maintaining RBC shape and deformability

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3
Q

what is the hereditary defect called in cell membrane?

A

hereditary spherocytosis

red cells spherical & removed from circulation

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4
Q

hereditary spherocytosis has defects in?

A 
ankyrin
alpha spectrin
beta spectrin
band 3
protein 4.2
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5
Q

hereditary spherocytosis presentation? (4)

A

anaemia
jaundice
splenomegaly
pigment gallstones

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6
Q

hereditary spherocytosis treatment?

A

folic acid
transfusion
splenectomy

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7
Q

why do a splenectomy?

A

as this is where RBC destroyed so they last longer but tried to avoid this

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8
Q

what can go wrong with the metabolic pathways of RBC?

A

glycolysis

pentose phosphate shunt (G6PD)

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9
Q

what is G6PD?

A

glucose 6 phosphate dehydrogenase

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10
Q

what does G6PD do?

A

protects red cell proteins from oxidative damage

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11
Q

how is G6PD linked?

A

X-linked

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12
Q

how does G6PD problem present?

A

anaemia
neonatal jaundice
splenomegaly

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13
Q

what can precipitate G6PD?

A

antimalarials
analgesics
anthelmintics

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14
Q

what is normal Hb A in adult haemoglobin?

A

Hb A: 97%

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15
Q

what happens to haemoglobin in Thalassaemia?

A

reduced or absent globin chain production

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16
Q

haemoglobin in sickle cell?

A

mutations leading to structurally abnormal globin chains

17
Q

what is sickle haemoglobin composed of?

A

haem molecule and 2 a-chains and 2-b (sickle) chains

18
Q

what does sickle cell lead to?

A 
red cell injury
cation loss
dehydration
haemolysis
vaso-occlusion
19
Q

how does sickle cell present?

A

painful vaso-occlusive crisis
stroke
infection
chest crisis

20
Q

how to treat sickle cell?

A

hydration
O2

AVOID PETHIDINE

21
Q

sickle cell management?

A 
vaccine
penicillin
folic acid
blood transfusion
BM transplant
22
Q

what is Thalassaemia?

A

reduced or absent globin chain production

23
Q

what happens in b-thalassaemia major?

A 
severe anaemia (3-6m age)
expanded ineffective BM
deformities
splenomegaly
growth retardation
24
Q

life expectancy in b-thalassaemia major?

A

less than 1 year

25
Q

treatment for b-thalassaemia major?

A

chronic transfusion support

26
Q

when does death often happen in b-thalassaemia major?

A

2nd or 3rd decade due to heart/liver/endocrine failure

27
Q

what can be used in b-thalassaemia major to increase life expectancy >40y?

A

iron chelation therapy

BM transplantation

28
Q

normal Hg in males?

A

140-180

116-156 >70y

29
Q

normal Hg in females?

A

120-160

108-143 >70

30
Q

what can be seen on blood smears in sickle cell due to lack of spleen?

A

howell jolly bodies

Haematology (8 decks)

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