Red Cells 1

Question 1

what is anaemia

Answer 1

reduction in red cells or their haemoglobin

Question 2

what are causes

Answer 2

blood loss
increased destruction
lack of production
defective production

Question 3

what are substances required for red cell production

Answer 3

metals
vitamins
hormones

Question 4

where does red cell breakdown occur

Answer 4

macrophages in sleepn
liver
lymph nodes
lungs 
bone arrow

Question 5

what is the life of a RBC

Answer 5

120 days

Question 6

what is globin used for

Answer 6

amino acids

Question 7

what is harm used for

Answer 7

iron recycled into haemoglobin

Question 8

what is the membrane stricture

Answer 8

like a bag

skeletal proteins are responsible for maintaining red cell shape and deformability

Question 9

what are examples of membrane defects

Answer 9

hereditary elliptcytosis
hereditary pyropoikolocytosis
ovalocytosis

Question 10

what is hereditary spherocytosis

Answer 10

autosomal dominant
cannot form proper shape of red cell
removed by haemolytic

Question 11

what are the defects in the proteins

Answer 11

ankyrin
alpha spectrin
beta spectrin
band b 
protein 4.2

Question 12

how does HS present

Answer 12

anaemia
jaundice
splenomegaly
gallstones

Question 13

what investigation would you do

Answer 13

reticulocyte
bilirubin
cooms test

Question 14

what is the treatment for HS

Answer 14

folic acid
transfusion
give nutrients
splenomegaly

Question 15

what does G6PD do

Answer 15

protects cels from oxidative damage
is X linked-affects males
blister cells

Question 16

what is the presentation of G6PD

Answer 16

degrees of anaemia
neonatal jaundice
splenomegaly
gallstones

Question 17

triggers to G6PD

Answer 17

infection
acute illness
drugs-antimalarials

Question 18

describe the structure of Haematology nd binding to 02

Answer 18

3 haem and 4 iron
more 02 binds, changes affintiy
increases affinity
increase in temp decreases 
decrease in ph decreases 
if more 02 haematology binds to it

Question 19

what is the structure of normal haemoglobin

Answer 19

4 alpha

2 beta

Question 20

what are the normal ranges for haemoglobin

Answer 20

male 12-70 120-180
male >70 116-156
female 12-70 120-160
female >70 108-143

Question 21

mutations that lead to reduced or absent chain prodcution

Answer 21

thalasseamia

Question 22

mutations leading to structurally abnormal global chain

Answer 22

sickle cell

Question 23

explain sickle cell

Answer 23

composed of 2 alpha chins and 1 beta sickle chain
autosomal recseeive
red cells being rigid and sticky

Question 24

what are consequences of SS

Answer 24

acute chest syrndome- endothelial activation
stroke
inflammation

Question 25

what are the presentation of SS

Answer 25

``` severe pain chest crisis stroke infection gallstones ```

Question 26

what is the management for sickle cell

Answer 26

``` bone marrow opiates hydration long life prophylaxis hydroxycarbamide ```

Question 27

what is thalasseamia

Answer 27

reduced or absent chain production | autosomal recessive

Question 28

what are the signs

Answer 28

expansion of bone marrow increased bone thickness bone defromities

Question 29

what is the treatmentr for thalasseamia

Answer 29

iron chelation therapy | oral deferasiox

Question 30

mutations that lead to reduced or absent chain prodcution

Answer 30

thalasseamia

Question 31

mutations leading to structurally abnormal global chain

Answer 31

sickle cell

Question 32

explain sickle cell

Answer 32

composed of 2 alpha chins and 1 beta sickle chain autosomal recseeive red cells being rigid and sticky

Question 33

what are consequences of SS

Answer 33

acute chest syrndome- endothelial activation stroke inflammation

Question 34

what are the presentation of SS

Answer 34

``` severe pain chest crisis stroke infection gallstones ```

Question 35

what is the management for sickle cell

Answer 35

``` bone marrow opiates hydration long life prophylaxis hydroxycarbamide ```

Question 36

what is thalasseamia

Answer 36

reduced or absent chain production | autosomal recessive

Question 37

what are the signs

Answer 37

expansion of bone marrow increased bone thickness bone defromities

Question 38

what is the treatmentr for thalasseamia

Answer 38

iron chelation therapy | oral deferasiox