HAEM 1 Flashcards
sites of hemopoiesis in embryo
at birth
in adults
yolk sac. liver. spleen 3-7 months
BM. liver and spleen when needed
adult: BM of skull, ribs, sternum, pelvic, proximal femur
how many globin/protein submits carry a single heam
when fully saturated 1g of Hb will bind how much o2
4
1.34ml
how much iron do we absorb each day
what is the plasma pool
where is iron stored
1g
4mg
in ferritin mainly in the liver
adult males HB and HCT
adult females
when does RBC production take place
<130 HCT 0.38-0.52
<120 0.37-0.47
BM - erythroid precursors cluster around a central histiocyte
reticulocytes are what
causes of decreased production (decrease retic count)
causes of increased loss/destruction (high retic count)
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
causes of genuine megalablastic macrocytic anaemia whats seen on the blood film what is a megaloblast other ix treatment
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
non megaloblastic causes of macrocytic anaemic
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
spurious causes of macrocytic anaemia
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
what does b12 and folate do
symptoms of defic
cause of b12 defic
cause of folate defic
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
treatment of b12 and folate defic where does b12 and folate come from body stored absorbed where daily requirement
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.
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
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
microcytic anaemia haem deficiency causes
global defic causes
decreased iron: iron def/chronic anaemia
porphyrin synthesis: lead poisoning, pyridoxine, responsive anaemias
congenital: siderblastic anaemia
thallasaemias
iron deficiency symptoms on Bfilm dietary causes and treatment blood loss causes malabsorption causes
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)
chronic anaemia is what
what is seen on a blood film
serum transferrin/IBC/ferratin levels
lack of available iron
normochronic/hypochronic normocytic anaemia
decreased serum and total iron brining capacity. normal or increased ferratin
sideroblastic anaemia symptoms
treatment
anaemia. increased L and S. increased iron = heart, kidneys and liver damage
supportive. transfuse if anaemic. iron chelation with desferioxamine
iron tests Hb
serum iron/transferrin/transferrin sat
serum ferritin
tranfserrin
ferratin
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
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
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
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
microcytic hypochromic anaemia. ineffective erythropoiesis and haemolysis
monogenic dx. malaria endemic areas
alpha chains affected. SEA, ME, meditirean
HbA HbA2 and HbF
only HbA
alpha thal trait how many missing
symp
ix
mistaken for what
1 or 2
asymp
microcytic hypochromic with mild anaemia
can be mistaken for iron def but normal ferritin and RBC count is up
HbH disease is what alpha chain production symp cx excess beta chain form what ix
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
Hb barts hydrops fetalis is what
symp
ix
cx
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
beta thal trait symp ix
beta thal intermediate symp rx
asymp. no/mild anaemia. HbA2 >3.5%. low MCV/MCH
mod severity requiring occasional transfusions
beta thal major ix symp require what and to keep what at what level endocrine cardiac liver secondary complication of treatment and management
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
diagnosis of thal
exclude iron defic
film: hypochromic target cells anisopoikoliocytes
HPLC: identifies abnormal Hb increased HbA2 in beta thal trait
for alpha trait need genetic testing
sickling disorders genetics what replaces what
what happens to Hb^s polymerase
sickle cell trait is what symp ix and cx of it
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
sickle cell anaemia ix thrombotic/vascoclosure crisis sequestration aplastic haemolytic
treatment for sickle cell crisis
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
chronic haemolsysi in sickle cell leads to what
hyposplenism
long term effects
shortened RBC lifespan
due to repeated splenic infarcts
impaired growth, sepsis risk, risk of organ damage
life long treatment of sickle cell
hyposplenism - proph penicillin
pneumococcal, haemophilia and meningococcal vaccination
FA
hydroxycarbimide to indue HbF production
sickle cell disease is what and ex
what is HbSc
sickle cell and another beta chain mutation: hbS/beta thal trait
milder but increased risk of thrombosis. lysine replaces glutamate
