questions

Question 1

what are immunoglobulins

Answer 1

produced by B-cells and plasma cells
each immunoglobulin recognises a specific antigen
can be expressed on B cell surface or released into blood stream as antibodies by plasma cells

Question 2

what are immunoglobulins made up of

Answer 2

2 heavy chains- determines the class of antibody produced
2 light chains
together the type of heavy and light chains are called isotype of the antibody

Question 3

common blood results in alcoholic liver disease

Answer 3

macrocytic anaemia and thrombocytopenia

Question 4

first line management of patient with autoimmune haemolytic anaemia

Answer 4

steroids

Question 5

basophilic stippling of red blood cells means

Answer 5

lead poising or siderblastic anaemia

Question 6

presentation of siderblastic anaemia

Answer 6

microcytic anaemia
fatigue
pallor
SOB

Question 7

where is folate absorbed

Answer 7

the jejunum

Question 8

causes of B12 deficiency

Answer 8

not enough dietary intake
pernicious anaemia
gastrectomy
malabsorbtion: crohn’s
medicine: metaformin

Question 9

causes of folate deficiency

Answer 9

diet
malabsorption: coeliac
excessive peeing: heart failure, liver damage, long term dialysis
medicine: anticonvuslants
pregnancy
malignancy
exfoliating dermatitis

Question 10

what is pernicious anaemia

Answer 10

autoimmune condition resulting in destruction of gastric parietal cells > intrinsic factor deficiency

Question 11

importance of B12 and folate

Answer 11

co-factors in DNA synthesis and nuclear maturation and DNA modification and gene activity

Question 12

how is B12 metabolised

Answer 12

freed from protein complexes in food by gastric enzymes
then binds to R binder (binding protein)
released from R binder by pancreatic enzymes and then binds to intrinsic factor
intrinsic factor carries B12 to specific receptor on mucosa of ileum
B12 inters ileal cells and IF is excreted

Question 13

how is folate absorbed

Answer 13

dietary folates converted to monoglutamate
absorbed in jejunum
stores are lower than for B12

Question 14

presentation of B12/folate deficiency

Answer 14

anaemia
weight loss
diarrhoea
infertility
sore tongue
jaundice
development problems

Question 15

why can patients with macrocytic anaemia appear jaundiced

Answer 15

due to ineffective erythropoiesis caused by intramedullary haemolysis
meaning a breakdown of cell within the bone marrow

Question 16

B12 deficiency specific symptoms

Answer 16

neurological problems
- posterior/dorsal column abnormalities
- neuropathy
- dementia
- psychiatric manifestations

Question 17

management of pernicious anaemia

Answer 17

vit B12 injections for life- hydroxocobalamin

Question 18

causes of microcytic anaemia

Answer 18

TAILS
- thalassaemia
- anaemia of chronic disease
- iron deficiency
- lead poising
- sideroblastic

Question 19

what causes anaemia of chronic disease

Answer 19

IL-6 is released due to chronic diseases and increases production of hepcidin > this down regulates ferroportin expression, reducing iron absorption and precipitating anaemia

Question 20

what is sideroblastic anaemia

Answer 20

excess iron buildup in mitochondria due to failure to incorporate iron into haem
can be hereditary

Question 21

most common type of Hodgkin’s lymphoma

Answer 21

nodular sclerosing

Question 22

bleeding and bruising, petechial and purpuric rash
often with a history of viral illness

Answer 22

immune thrombocytopenic purpura

Question 23

management of immune thrombocytopenic purpura

Answer 23

prednisolone
IV immunoglobulins
blood transfusion
platelet transfusions only work temporarily

Question 24

management of thrombotic thrombocytopenic purpura

Answer 24

IV plasma exchange
splenectomy

Question 25

what does cryoprecipitate contain

Answer 25

factor VIII, fibrinogen, von Willebrand factor and factor XIII

Question 26

when is hydroxyurea used in sickle cell disease

Answer 26

to prevent a further crisis if the patient has had repeated attacks

Question 27

diagnostic of beta thalassaemia trait

Answer 27

raised HbA2

Question 28

presentation of beta thalassaemia major

Answer 28

presents aged 6-24 months (as HbF falls)
pallor
failure to thrive
hepatosplenomegaly
skeletal changes
organ damage

Question 29

haemoglobin analysis of beta thalassaemia major

Answer 29

mainly HbF
no HbA

Question 30

management of beta thal major

Answer 30

regular transfusion programme to maintain Hb
- risk of iron overload from transfusion
bone marrow transplant may be option

Question 31

consequences of iron overload

Answer 31

endocrine dysfunction: impaired growth and pubertal development, diabetes, osteoporosis
cardiac disease: cardiomyopathy, arrhythmias
liver disease: cirrhosis, hepatocellular cancer

Question 32

management of iron overload

Answer 32

250mg of iron per unit packed red cells
chronic anaemia also drives increased iron absorption
iron chelating drugs: desferrioxamine

Question 33

what type of haemophilia is turners related to

Answer 33

haemophilia A
- because it is X linked recessive and people with turners only have one X chromosome