Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Year 3 Haematology > Red cells 1 - congenital anaemias > Flashcards

Red cells 1 - congenital anaemias Flashcards

1
Q

What is anaemia?

A

reduction in red cells or Hb content

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Main substances required for RBC synthesis

A

erythropoietin
Vit B 12 and folic acid
iron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

life span of RBC

A

120 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

reticuloendothelial system

A

macropahges in spleen, liver, lung, LN etc

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

recycling of RBC

A

globin - amino acids

haem - iron and bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

unconjugated bilirubin

A

bound to albumin in plasma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

3 areas of problems in congenital anaemias of RBC

A

membrane
enzymes
haemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

red cell membrane

A

skeletal proteins keep shape and deformity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

red cell membrane proteins

A

ankyrin
beta and alpha spectrin
band 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

inheritance of hereditary spherocytosis

A

autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

RBC in hereditary spherocytosis

A

defects in membrane proteins

RBC are spherical and removed by RE system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Clinical presentation of hereditary spherocytosis

A

anaemia
jaundice (neonatal)
pigment gallstones
splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Treatment of hereditary spherocytosis

A

folic acid
transfusion
splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

G6P-D

A

enzyme protecting from oxidative stress

NADPH reduce glutathione, detoxifies oxygen species

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What does G6P-D deficiency confer an advantage for?

A

malaria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

G6P-D deficiency inheritance

A

x-linked

affect males and female carriers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

G6P-D deficiency blood film

A

blister and bite cells

18
Q

clinical presentation of G6P-D deficiency

A

neonatal jaundice
Drugs, beans,infection precipitated
splenomegaly
pigment gallstones

19
Q

precipitants of G6P-D deficiency

A

drugs eg anti-malarials,anti-bacterials

fava beans

20
Q

structure of haemoglobin

A

iron containing haem with 4 globin chains - 2 alpha and 2 beta

21
Q

haemoglobin oxygen binding

A

oxyhaemoglobin - relaxed binding

deoxyhaemoglobin - tight binding : 2,3-DPG

22
Q

Oxygen dissociative curve

A

hyperthermia
acidosis
hypercapnia

23
Q

HbA or HbF higher O2 affinity?

A

HbF

24
Q

alpha and beta chains - how many genes?

A

2 alpha chains - 4 alpha genes

2 beta chains - 2 beta genes

25
Q

Thalassaemia underlying problem

A

reduced or absent globin chain production

26
Q

sickle cell underlying problem

A

severely abnormal globin chain

27
Q

sickle cell inheritance

A

autosomal recessive

1 in 4 affected, 1 in 2 carrier

28
Q

Defomity of sickle cell

A

haem + 2 alpha chains + 2 beta (sickle chains)

point mutation in beta chain

29
Q

Consequences of sickle cell

A

haemolysis due to red cell injury
inflammation, vasomotor tone, endothelial activation
VASO-OCCLUSION

30
Q

Clinical presentation of sickle cell

A 
painful vaso-occlusive crisis (bone)
stroke
chest crisis 
increased infection due to hyposplenism 
sequestration crisis - spleen, liver 
chronic haemolytic anaemia
31
Q

life expectancy with sickle cell

A

M=42, female = 48

due to child and perinatal mortality

32
Q

Painful crisis treatment

A

opiates
oxygen
hydration
?antibiotic

33
Q

chest crisis symptoms

A

increasing hypoxia, chest pain, fever, infiltrates on CXR

34
Q

chest crisis treatment

A 
resp support 
antibiotics 
IV fluids 
analgesia 
transfusion
35
Q

life long prophylaxis for sickle cell

A

vaccination, penicillin (+malaria), folic acid

36
Q

acute events management for sickle cell

A

hydration, oxygen, infection, analgesia

37
Q

Other treatments for sickle cell

A

transfusion
disease modifying drugs - hydroxycarbamide
bone marrow transplant and gene therapy

38
Q

4 types of thalassaemia

A

homozygous alpha zero - incompatible with life
beta thalassaemia major - life long transfusion
non transfusion dependent
thalassaemia minor

39
Q

Thalassaemia symptoms

A

severe anaemia:3-6months old
expansion of ineffective bone marrow - hair on end
splenomegaly
growth retardation

40
Q

Treating thalassaemia

A

chronic transfusion support (4-6weekly)
avoid iron overload - chelation
curative bone marrow transplant

41
Q

outcome of iron overload

A

heart, endocrine, liver failure

Year 3 Haematology (18 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions