haem Flashcards
(120 cards)
1
Q
what are the sites of haemoposiesis:
- embryo
- at birth
- adult
A
embryo - yolk sace then liver then marrow
at birth- mostly marrow
adult- bone restirted to skull, rib sternum, pelvis and prox femur
2
Q
what do erythroid precursors develop into
A
platelets and RBCS
3
Q
what do monocyte precursors develop into
A
macrophages, and granulocytes eg neut, baso,eosino
4
Q
whta cells are also know as polymorphs
A
neutrophils
5
Q
what graulocyte has a bi-lobes nucelus
A
eosinophil
6
Q
what does the nucleus of a neutrophil look like
A
multi-loculated
7
Q
describe the makeup of a haemoglobin molecule
A
2 alpha chanins
2 bets chains
haem group (FE2+) in porphyrin ring in each chain
8
Q
bilirubin is created from the breakdown of what group
A
porphyrin ring
9
Q
what is glbin recycled to
A
amino acids
10
Q
how do red cells creat energy
A
only via glycolysis - 2 ATP generated
11
Q
what type of iron can oxygen bind to
A
Fe2+ not Fe3+
12
Q
how is FE2+ converted back to FE3+ in the RBC
A
via NADP - redox reaction - gain electron
13
Q
function of G6PD enxyme
A
prevents oxidtive stress in RBC
14
Q
how is G6PD deficiency inherited
A
X-linked recessive
15
Q
how is carbon dioxide trasnported in the blood
A
- 10% dissolved
30% - bound to Hb as carbimo - Hb
60% as bicarb
16
Q
what shifts bohr effect curve to the right
A
increased CO2, increased H+ (low pH)
2-3 BPG
17
Q
does macro or microcytic anaemia casue hypochromia
A
microcytic
18
Q
causes of microcytic anaemia
A
T- thalaaseami
A- anaemia of chronic disease
I -iron deficiency
L- lead poisioning
S- sideroblastic
19
Q
in microcytic anaemia pathologically where does the defect ccur
A
in heamoglobin production - haem or globin deficiency
therefore cytoplasmic defect
20
Q
condition that causes a globin deficiency
A
thalassaemia
21
Q
what is circulating iron bound to
A
trasnferrin
trasnports iron to marrow
22
Q
what is iron stored as and where
A
ferritin in liver mainly - and macrophages
23
Q
what would transferrin saturations be in anaemia of Cd and iron deficiency
A
reduced
24
Q
where is iron absorved
A
jejunum
25
cutanoeous features of iron deifciency anaemia
itchy rash
koiionychia
angular chelitis
smooth tongue
26
treatment of iRon deficiency anaemia and expected response
ferrous fumurate - shoud rise about 10 g/L a week
27
how to differetiate between a megaloblastic anaemia and non- megaloblastic anaemai
the presence of hypersegmented neutrophils (>5) indicates megaloblastic i.e impaired DNA synthesisi
28
how to dsitinguish between B12 and folated deficiency
methymalonic acid - increased in b12
29
causes of megalblastic anaemia
b12 deficency
folate deficiency
DNA affetign drugs- methotrexate, azathioprine , sulfa derivatives eg salufasalazine and trimepthoprim , phenytoin and 5-fluorouracil
30
causes of b12 deficiency
vegans
pernicious anaemia
31
causes of folate deficiency
diet malabsorption
excessive utilisation eg, pregnancy, haemlysis, anti-convulsants
32
where is B12 absorbed and how long is it stored for
ileum and 2-4 years
33
where is folate absorbed and how long is it stroed for
duodenum/jejunum and 4 months
34
Symptoms of b12 and folate deficiency
signs of anaemia and fatigue
weight loss, diarrhoea, inferitlity
sore tongue- burning mouth and jaundice
neuro symptoms
35
what would be seen on blood film in megaloblastic anaemia
hypersegmented neutrophils
macro-ovalocytes
36
causes of non -megaloblastic anaemia
alcohol
preganncy
liver disease
hypothyroid
marrow failure- eg myledysplasia, myeloma and aplastic anaemia
37
causes of normocytic anaemia
anaemia of chronic disease
chronic kidney disease
aplastic anaemia
haemolytic anaemia
acute blood loss
38
what types of cells would be seen on blood film in iron deficiency anaemia
anisopoikilocytosis (red blood cells of different sizes and shapes) ,
target cells,
'pencil' poikilocytes
39
what age demographic has the highest incidence of iron deficiency anameia
pre-school children due to preiods of rapid growth- high iron demand
40
what chromosome is the ABO blood grouping found on
chromosome 9
41
what antigen and antibody does blood group A have
A antigen and Antibody B
42
what antigens does blood group O have
neither - has both antibodies
43
what Ig are blod group antibodies
IgM
44
what HB levels indicate trasnfusion
70g/l
80 in ACS
45
a platelet count of what in active bleedign would indicated plateelt trasnfusion
<30 if grade 2 eg haematemesisi
<100 if critical sites eg CNS
46
what type of blood product transfusion has the higehst level of bacterial infection
plateleet
47
does ahigh or low INR increase blleding rise
high - higher the thinner
48
when should FFP be given
if PT or APTT>1.5
49
what is the universal doner of FFP
AB as lacks antibodies
50
what is found in FFp
clotting factors, albumin and immunoglobulin.
51
what is irradiated blood missign
depleted of t-cell lymphocytes
52
What does the use of irradiated blood products avoid
transfusion assoc grafte versus host disease
53
describe the 3 main forms of haemoglobin
HbA - 2aplha 2 beta
HbA2 - 2 alpha and 2 delta
HbF - 2 alpha 2 gamma
54
alpha globin chains are found on what chromosome and how many copies
chromo 16
2 alpha genes per chromo
4 in total
55
beta globin genes are found on what chromosome and how many copies
chromo 11
one copy per chromo
56
when are adult level beta chains reached
6-12 months
57
what is difference between thalassaemi and sickle cell
thala - decreased rate of globin synthesis - normal chains
sickle - norm rate - abnorm chains
58
in alpha thalassaemia if only 1 or 2 copies are missing hwo would this present
thalassaemia trait - microcytic, hypochromic red cells but norm Hb - asymp carrier
59
if 3 copies of alpha globin allele affected in alpha thalassaemia whta is this called and how would it present
HbH -
jaundice splenomegaly growth retardation
hypochromic microcytic anaemia
60
if all 4 alpha globin genes affected what thalassaemia would this cause
Hb bart/ hydrops fetalis - death in utero
61
in Hb H disease what is seen on blood film and why
golf ball cells - excess beta chain form tetramers
hypochromic microcytic anaemia
62
what type of mutation casues alpha thalassaemia
deleteion
63
what type of mutation causes beta thalassaemia
point mutation
64
how are teh thalassaemia inherited
auto recessive
65
raised what is diagnostic of beta -thalassaemia trait and why
HbA2 - 2 alh , 2 delta chians
66
how does beta thalassaemi amajor present
presents in the first year of life with failure to thrive and hepatosplenomegaly
extramedullary haematopoesisi casues :
- hepatosplenomegaly
- skeletal changes
- organ damage
67
what would be seen on Hb analysis in beta-thalassaemia major
HbF and HbA2 raised
NO HBA
68
management of beta-thalassaemia major
repeated transfusion- 95-105
this leads to iron overload → organ failure
iron chelation therapy is therefore important (e.g. desferrioxamine)
69
genetic mutation in sickle cell
point mutation in codon 6 of B globin gene
turns glutamine into valine
therefore HbS
70
what is sickle cells trait
one abnormal sick cell gene one normals
aymp carrier unless in crisis
71
inheritance patter on sickle cell
auto recessive
72
who tends to get sickle cell and why
afrocarribean - protective against malaria
73
what age does sickle cell anaemia present and why
4-6 months when HbF stops
74
inbestigation for sickle cell
plasmaphoresis
75
precipitants of sickle cell crisi
cold, infection. dehydration , deoxygenation
76
how is a sickle cell thrombotic crisis diagnosed
clinically
77
what casues an aplastic crisis in sickle cell and what is seen
parvovirus
sudden fall in Hb and reduced retic count
78
management of sickle cells anaemia
penicillin if hypospelinism
hydroxycarbamide/hydroxyurae - induce HBF production
pneumo vaccine every 5 years
79
treatment of a Non-haemolytic febrile reaction to transfusion
slow rate
monitor
paracetamol
80
cause of transfusion reaction anaphylaxis and tx
patients with IgA deficiency who have anti-IgA antibodies
Stop the transfusion
IM adrenaline
ABC support
oxygen
fluids
81
pathophysiology of acute heamolytic trasnfusion reaction
red blood cell destruction by IgM-type antibodies.
82
do reticulocytes have a nucleus
no only RNA not DNA
83
what conditions cause MAHA
DIC
HUS
TTP
84
what antibodies are involves in RBC destruction in paroxysmal cold haemoglobinuria and paroxysmal nocturnal haemoglobinuria
IgG
85
examples of immune mediated intravasculaar haemlysis
cold haemoglobinuria
86
what type of haemolysis is osmotic lysis
intravascular - hypotonic Iv solution causes RBCs to burst
87
what is produced in liver if high iron stroes to down regulate absorption
hepcidin- also produced in anaemia of chronic disease
88
primary vs secondary haemostasis
primary - platelet plug
secondary - coagulation cascade
89
in primary haemostasis endothelial damaage causes exposure of what factors
collage and VW factor
90
Pt and APTT what one is intrinsic pathway and what one is extrinsic
intrinsic - PT
extrinsic - PATT
91
what would cause multiple clotting factor deficiency
DIC And liver failure
92
vitamin k dependent clotting factors
2,7,9 and 10
93
what do warfarin adn heparin affect with regards to PT and APTT
warfarin - PT time
heparin - APTT
94
broad cateogries of casues of failure of platelet plug and what they include
VASCULAR - collagen deficiency eg marfans
vasculitis- HSP
scurvy - vit c
PLATELETS - thrombocytopenia
hereditary vs acquires
eg dic, ITP (autoimmune), hypersepnism
VW deficiency
95
casues of failure of secondary haemostasis - fibrin clot
clottin gfactor deficiency
multiple- DIC , liver disease
single - haemophilia
96
how might pbstructive jaundice casue clotting factor deficiency
bile needed to absorb vit k
97
treatment of ITP in adults
steroids
98
inheritance pattern of haemophilia
x-linked recessive
99
difference between haemophilia A and B
A - VIII deficiency
B - IX deficiency (christmas disease)
100
PT and APTT in haemophilia
PT - norm
APTT - prolonged
101
what breaks dwon the fibrin clot
plasmin
102
role of tpa in body
tissue plasminogen actovator converts plasminogen to plasmin - this breaks dwon fibrin clot
103
what are the naturally occuring anti-coagulatns
antithrombin,
protein C and S
104
examples of inherited thrombophilias
factor V leiden
protein s deficiency
preotein c deficiency
anti thrombin deficicency
prothrombin 20210 mutation
105
example of an acquired thrombophili
anti-phospholipid syndrome
106
features of anti phospholipid
arterial and venous thrombosis
fetal loss
mild thrombocytopenia
107
what is seen with Pt and APTT in anti-phospholipid
normal PT
prolonged APTT
108
antibodies seen in anti-phospholipid
anticardiolipin antibodies
anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
lupus anticoagulant
109
management of anti-phospholipid
prophylaxis - asprin
after first event - warfarin
110
MOA of heparin
potentiate anti thrombin
111
diff between unfractioned and LMWH MOA's
unfraction- anti-thrombin binds with thrombin
LMWH - anti thrombin binds with factor Xa
112
what form of heparin requires monitoring adn how
unfractionated via APTT
113
heparin reversal agent
protamine sulphate
114
side effects of heparin
osteoporosis
trombocytopenia
bleeding
hyperkalaemia
115
side effects of heparin induced thrombocytopenia
pro-thrombotic
116
what juice is avoided with warfarin
cranberry
117
why is heparin used alongside warfarin in the first 5 days
to avoid skin necrosis
when warfarin is first started biosynthesis of protein C is reduced- this results in a temporary procoagulant state after initially starting warfarin,
118
what is required for vitamin K absorption
bile
119
why are newborns given vitamin K
to avoid haemorrhagic disease of the newborn
120
lifespan of plateele
7-10 days
