HAEM 1

Question 1

sites of hemopoiesis in embryo
at birth
in adults

Answer 1

yolk sac. liver. spleen 3-7 months
BM. liver and spleen when needed
adult: BM of skull, ribs, sternum, pelvic, proximal femur

Question 2

how many globin/protein submits carry a single heam

when fully saturated 1g of Hb will bind how much o2

Answer 2

4

1.34ml

Question 3

how much iron do we absorb each day
what is the plasma pool
where is iron stored

Answer 3

1g
4mg
in ferritin mainly in the liver

Question 4

adult males HB and HCT
adult females
when does RBC production take place

Answer 4

<130 HCT 0.38-0.52
<120 0.37-0.47
BM - erythroid precursors cluster around a central histiocyte

Question 5

reticulocytes are what
causes of decreased production (decrease retic count)
causes of increased loss/destruction (high retic count)

Answer 5

larger than normal still have DNA stain purple/deep red. on blood film they are polychormatic

hypoprliferation
maturation abnormality: impaired Hb, impaired cell division, ero isn’t being produced

bleeding haemolysis

Question 6

causes of genuine megalablastic macrocytic anaemia
whats seen on the blood film 
what is a megaloblast
other ix
treatment

Answer 6

B12 defic, folate defic, drugs, rare inherited causes
macroocalocytes and hyperhsegmented neutrophils
abnormal large uncleared RC precurosr with an immature nucleus
assay b12 and folate. anti GPC/Anti IF
treat cause. FA 5mg/day. if life threatening - tranfuse

Question 7

non megaloblastic causes of macrocytic anaemic

Answer 7

alcohol liver disease and hypothyroidism - not associated with anaemia - due to changes in red cell
marrow failure: myelodysplasia, myeloma, aplastic anaemia - is associated with anaemia

Question 8

spurious causes of macrocytic anaemia

Answer 8

acute blood loss of haemolsyss leading to increased reticulocytes with increased MCV
cold agglutins: AI disease against RBC causing them to clump together leading to increased MCV

Question 9

what does b12 and folate do
symptoms of defic
cause of b12 defic
cause of folate defic

Answer 9

enable chemical reactions that provide enough nucleotides for DNA synthesis

anaemia. decreased weight. diarrhoea. sore tongue. jaundice. developmental problems. neuro - myeline sheath (just b12) can be there without haem findings
coliac. IBD. chronic pancreatitis. vegans. bypass
dietary. IBD. haemolysis. exfoliating dermatitis. pregnancy. malignancy. anti convulsants

Question 10

treatment of b12 and folate defic 
where does b12 and folate come from 
body stored 
absorbed where
daily requirement

Answer 10

diet. FA replacement. hydrocarlbumin for neuro symptoms.
animal. leafy veg, yeast destroyed by cooking
2-4y. 4 months
ileum. D & J
1-3 mcg. 100mcg.

Question 11

pernicious anaemia is what 
what else is it associated with 
what is b12 needed for 
what does it lead to - pathology 
symptoms 
ix
predisposes to what 
treatment

Answer 11

AI destruction of gastric parietal cells
atrophic gastritis, other AI disease
needs for division of cells
ineffective erythropoiesis. RC will die prematurely in BM.
mildly jaendicie due to intramedullary haemolysis
Anti IF specific not sensitive > anti GPC
gastric cancer
hydroxycobalmamin alt days IM 1g for 6 weeks then 3 monthly for life

Question 12

microcytic anaemia haem deficiency causes

global defic causes

Answer 12

decreased iron: iron def/chronic anaemia
porphyrin synthesis: lead poisoning, pyridoxine, responsive anaemias
congenital: siderblastic anaemia

thallasaemias

Question 13

iron deficiency symptoms 
on Bfilm
dietary causes and treatment 
blood loss causes
malabsorption causes

Answer 13

koilonychia, atrophic glossitisit, post cricoid webs, angular stomatitis
target cells, pencil porkilocytes

relative in pregnancy. absolute in vegans
diet. ferrous sulphate iron should go up 1g per week

mennohrgia >60ml = >30mg of iron los. GI - tumours, NSAIDs, ulcers. haematuria

colic. acholydria (absence of decreased production of HCl)

Question 14

chronic anaemia is what
what is seen on a blood film
serum transferrin/IBC/ferratin levels

Answer 14

lack of available iron
normochronic/hypochronic normocytic anaemia
decreased serum and total iron brining capacity. normal or increased ferratin

Question 15

sideroblastic anaemia symptoms

treatment

Answer 15

anaemia. increased L and S. increased iron = heart, kidneys and liver damage
supportive. transfuse if anaemic. iron chelation with desferioxamine

Question 16

iron tests Hb
serum iron/transferrin/transferrin sat
serum ferritin

tranfserrin

ferratin

Answer 16

function iron
transported iron
storage iron

protein with 2 binding sites for iron. transport iron from donor tissues -> erythroid marrow.
decreases in iron def anaemia of chronic disease and increases in haemachromatosis

indirect measure of storage iron, each protein holds 4000 ferric ions
decreases in iron def

Question 17

foetal Hb
adult Hb
when are adult levels reached 
where are the alpha chains 
where are the metal chains 
what are hbg and what are the different types and ix

Answer 17

HbF alpha2 and gamma2
HbA2 alpha 2 weird gamma thing 2 HbA alpha 2 beta2
6-12 months of age
chrom 16 4 per cell. 11 2 per cell

hereditary, usually AR. thallasaemias. structural Hb variants.
antenatal screening. newborn screening. sometimes prenatal screening where appropriate

Question 18

thal is what what does it cause 
commonest what 
where is it more common 
what is alpha thal 
where is that more common 
what chains are affected 
what chains are affected in beta thal

Answer 18

microcytic hypochromic anaemia. ineffective erythropoiesis and haemolysis
monogenic dx. malaria endemic areas

alpha chains affected. SEA, ME, meditirean
HbA HbA2 and HbF

only HbA

Question 19

alpha thal trait how many missing
symp
ix
mistaken for what

Answer 19

1 or 2
asymp
microcytic hypochromic with mild anaemia
can be mistaken for iron def but normal ferritin and RBC count is up

Question 20

HbH disease is what 
alpha chain production 
symp
cx
excess beta chain form what 
ix

Answer 20

only 1 left
<30% of normal
anaemia w decreased MCV/MCH. splenomegaly. jaundice. growth retardation. gallstones. iron overload
acute haemolysis and aplastic crisis risk
form tetramas b4 that can’t carry o2
red cell inclusion bodied HbH seen with some stains

Question 21

Hb barts hydrops fetalis is what
symp
ix
cx

Answer 21

minimal or no alpha chain production, hbA can’t be made. HbBarts which is g4 or HbH majority at birth

severe anaemia. heart failure. growth retardation. oedema. hepatosplenomegay. skeletal abnormalities.

nucleated RBC in peripheral blood

most die in utero

Question 22

beta thal trait symp ix

beta thal intermediate symp rx

Answer 22

asymp. no/mild anaemia. HbA2 >3.5%. low MCV/MCH

mod severity requiring occasional transfusions

Question 23

beta thal major 
ix
symp
require what and to keep what at what level 
endocrine
cardiac
liver
secondary complication of treatment and management

Answer 23

no beta cell chain production

severe anaemia. reticulocytes. decreased MCV/MCH
film: macrocytosis, hypochromia, ankispoikilocytosis, target cells. HPLC: mainly HbF present. small amounts of HbA

6-24m. failure to thrive. allow. extra medullary haemopoesis. hepatosplenomegaly. skeletal changes. organ damage. cord compression

reg transfusions maintain Hb 95-105.

impaired growth, DM, osteoporosis
CM, arrthymias
cirrhosis, cancer

250mg of iron per unit of RBC - iron overload. desferioxamone

Question 24

diagnosis of thal

Answer 24

exclude iron defic
film: hypochromic target cells anisopoikoliocytes
HPLC: identifies abnormal Hb increased HbA2 in beta thal trait
for alpha trait need genetic testing

Question 25

sickling disorders genetics what replaces what
what happens to Hb^s polymerase
sickle cell trait is what symp ix and cx of it

Answer 25

point mutations in codon 6 of the beta global gene. substitutes glutamine with valine leading to HbS. HbS polymerase if exposed to low levels of oxygen for a prolonged period of time will distort i.e. sickle

one normal beta one abnormal
asymp carrier state
BFilm is normla. mainly HbA HbS <50%
may sickle in severe hypoxia like high amplitude of under GA

Question 26

sickle cell anaemia ix
thrombotic/vascoclosure crisis
sequestration
aplastic
haemolytic

treatment for sickle cell crisis

Answer 26

HbS >80%. no HbA

infection, dehydration, deoxygenation. infarcts in various organs

sickling in lungs/spleen causes pooling of blood making anaemia worse and can lead to things like acute chest syndrome

parvovirus
sudden decrease in Hb

rare. decreased Hb due to haemolsysis

opiate analgesia. hydration. rest. oxygen, Abs if infection, transfusion

Question 27

chronic haemolsysi in sickle cell leads to what
hyposplenism
long term effects

Answer 27

shortened RBC lifespan
due to repeated splenic infarcts
impaired growth, sepsis risk, risk of organ damage

Question 28

life long treatment of sickle cell

Answer 28

hyposplenism - proph penicillin
pneumococcal, haemophilia and meningococcal vaccination
FA
hydroxycarbimide to indue HbF production

Question 29

sickle cell disease is what and ex

what is HbSc

Answer 29

sickle cell and another beta chain mutation: hbS/beta thal trait

milder but increased risk of thrombosis. lysine replaces glutamate