haem Flashcards

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

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2
Q

what do erythroid precursors develop into

A

platelets and RBCS

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3
Q

what do monocyte precursors develop into

A

macrophages, and granulocytes eg neut, baso,eosino

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4
Q

whta cells are also know as polymorphs

A

neutrophils

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5
Q

what graulocyte has a bi-lobes nucelus

A

eosinophil

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6
Q

what does the nucleus of a neutrophil look like

A

multi-loculated

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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

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8
Q

bilirubin is created from the breakdown of what group

A

porphyrin ring

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9
Q

what is glbin recycled to

A

amino acids

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10
Q

how do red cells creat energy

A

only via glycolysis - 2 ATP generated

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11
Q

what type of iron can oxygen bind to

A

Fe2+ not Fe3+

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12
Q

how is FE2+ converted back to FE3+ in the RBC

A

via NADP - redox reaction - gain electron

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13
Q

function of G6PD enxyme

A

prevents oxidtive stress in RBC

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14
Q

how is G6PD deficiency inherited

A

X-linked recessive

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15
Q

how is carbon dioxide trasnported in the blood

A
  • 10% dissolved
    30% - bound to Hb as carbimo - Hb
    60% as bicarb
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16
Q

what shifts bohr effect curve to the right

A

increased CO2, increased H+ (low pH)
2-3 BPG

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17
Q

does macro or microcytic anaemia casue hypochromia

A

microcytic

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18
Q

causes of microcytic anaemia

A

T- thalaaseami
A- anaemia of chronic disease
I -iron deficiency
L- lead poisioning
S- sideroblastic

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19
Q

in microcytic anaemia pathologically where does the defect ccur

A

in heamoglobin production - haem or globin deficiency
therefore cytoplasmic defect

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20
Q

condition that causes a globin deficiency

A

thalassaemia

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21
Q

what is circulating iron bound to

A

trasnferrin
trasnports iron to marrow

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22
Q

what is iron stored as and where

A

ferritin in liver mainly - and macrophages

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23
Q

what would transferrin saturations be in anaemia of Cd and iron deficiency

A

reduced

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24
Q

where is iron absorved

A

jejunum

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25
Q

cutanoeous features of iron deifciency anaemia

A

itchy rash
koiionychia
angular chelitis
smooth tongue

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26
Q

treatment of iRon deficiency anaemia and expected response

A

ferrous fumurate - shoud rise about 10 g/L a week

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27
Q

how to differetiate between a megaloblastic anaemia and non- megaloblastic anaemai

A

the presence of hypersegmented neutrophils (>5) indicates megaloblastic i.e impaired DNA synthesisi

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28
Q

how to dsitinguish between B12 and folated deficiency

A

methymalonic acid - increased in b12

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29
Q

causes of megalblastic anaemia

A

b12 deficency
folate deficiency
DNA affetign drugs- methotrexate, azathioprine , sulfa derivatives eg salufasalazine and trimepthoprim , phenytoin and 5-fluorouracil

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30
Q

causes of b12 deficiency

A

vegans
pernicious anaemia

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31
Q

causes of folate deficiency

A

diet malabsorption
excessive utilisation eg, pregnancy, haemlysis, anti-convulsants

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32
Q

where is B12 absorbed and how long is it stored for

A

ileum and 2-4 years

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33
Q

where is folate absorbed and how long is it stroed for

A

duodenum/jejunum and 4 months

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34
Q

Symptoms of b12 and folate deficiency

A

signs of anaemia and fatigue
weight loss, diarrhoea, inferitlity
sore tongue- burning mouth and jaundice
neuro symptoms

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35
Q

what would be seen on blood film in megaloblastic anaemia

A

hypersegmented neutrophils
macro-ovalocytes

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36
Q

causes of non -megaloblastic anaemia

A

alcohol
preganncy
liver disease
hypothyroid
marrow failure- eg myledysplasia, myeloma and aplastic anaemia

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37
Q

causes of normocytic anaemia

A

anaemia of chronic disease
chronic kidney disease
aplastic anaemia
haemolytic anaemia
acute blood loss

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38
Q

what types of cells would be seen on blood film in iron deficiency anaemia

A

anisopoikilocytosis (red blood cells of different sizes and shapes) ,
target cells,
‘pencil’ poikilocytes

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39
Q

what age demographic has the highest incidence of iron deficiency anameia

A

pre-school children due to preiods of rapid growth- high iron demand

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40
Q

what chromosome is the ABO blood grouping found on

A

chromosome 9

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41
Q

what antigen and antibody does blood group A have

A

A antigen and Antibody B

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42
Q

what antigens does blood group O have

A

neither - has both antibodies

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43
Q

what Ig are blod group antibodies

A

IgM

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44
Q

what HB levels indicate trasnfusion

A

70g/l
80 in ACS

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45
Q

a platelet count of what in active bleedign would indicated plateelt trasnfusion

A

<30 if grade 2 eg haematemesisi
<100 if critical sites eg CNS

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46
Q

what type of blood product transfusion has the higehst level of bacterial infection

A

plateleet

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47
Q

does ahigh or low INR increase blleding rise

A

high - higher the thinner

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48
Q

when should FFP be given

A

if PT or APTT>1.5

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49
Q

what is the universal doner of FFP

A

AB as lacks antibodies

50
Q

what is found in FFp

A

clotting factors, albumin and immunoglobulin.

51
Q

what is irradiated blood missign

A

depleted of t-cell lymphocytes

52
Q

What does the use of irradiated blood products avoid

A

transfusion assoc grafte versus host disease

53
Q

describe the 3 main forms of haemoglobin

A

HbA - 2aplha 2 beta
HbA2 - 2 alpha and 2 delta
HbF - 2 alpha 2 gamma

54
Q

alpha globin chains are found on what chromosome and how many copies

A

chromo 16
2 alpha genes per chromo
4 in total

55
Q

beta globin genes are found on what chromosome and how many copies

A

chromo 11
one copy per chromo

56
Q

when are adult level beta chains reached

A

6-12 months

57
Q

what is difference between thalassaemi and sickle cell

A

thala - decreased rate of globin synthesis - normal chains
sickle - norm rate - abnorm chains

58
Q

in alpha thalassaemia if only 1 or 2 copies are missing hwo would this present

A

thalassaemia trait - microcytic, hypochromic red cells but norm Hb - asymp carrier

59
Q

if 3 copies of alpha globin allele affected in alpha thalassaemia whta is this called and how would it present

A

HbH -
jaundice splenomegaly growth retardation
hypochromic microcytic anaemia

60
Q

if all 4 alpha globin genes affected what thalassaemia would this cause

A

Hb bart/ hydrops fetalis - death in utero

61
Q

in Hb H disease what is seen on blood film and why

A

golf ball cells - excess beta chain form tetramers
hypochromic microcytic anaemia

62
Q

what type of mutation casues alpha thalassaemia

A

deleteion

63
Q

what type of mutation causes beta thalassaemia

A

point mutation

64
Q

how are teh thalassaemia inherited

A

auto recessive

65
Q

raised what is diagnostic of beta -thalassaemia trait and why

A

HbA2 - 2 alh , 2 delta chians

66
Q

how does beta thalassaemi amajor present

A

presents in the first year of life with failure to thrive and hepatosplenomegaly
extramedullary haematopoesisi casues :
- hepatosplenomegaly
- skeletal changes
- organ damage

67
Q

what would be seen on Hb analysis in beta-thalassaemia major

A

HbF and HbA2 raised
NO HBA

68
Q

management of beta-thalassaemia major

A

repeated transfusion- 95-105
this leads to iron overload → organ failure
iron chelation therapy is therefore important (e.g. desferrioxamine)

69
Q

genetic mutation in sickle cell

A

point mutation in codon 6 of B globin gene
turns glutamine into valine
therefore HbS

70
Q

what is sickle cells trait

A

one abnormal sick cell gene one normals
aymp carrier unless in crisis

71
Q

inheritance patter on sickle cell

A

auto recessive

72
Q

who tends to get sickle cell and why

A

afrocarribean - protective against malaria

73
Q

what age does sickle cell anaemia present and why

A

4-6 months when HbF stops

74
Q

inbestigation for sickle cell

A

plasmaphoresis

75
Q

precipitants of sickle cell crisi

A

cold, infection. dehydration , deoxygenation

76
Q

how is a sickle cell thrombotic crisis diagnosed

A

clinically

77
Q

what casues an aplastic crisis in sickle cell and what is seen

A

parvovirus
sudden fall in Hb and reduced retic count

78
Q

management of sickle cells anaemia

A

penicillin if hypospelinism
hydroxycarbamide/hydroxyurae - induce HBF production
pneumo vaccine every 5 years

79
Q

treatment of a Non-haemolytic febrile reaction to transfusion

A

slow rate
monitor
paracetamol

80
Q

cause of transfusion reaction anaphylaxis and tx

A

patients with IgA deficiency who have anti-IgA antibodies

Stop the transfusion

IM adrenaline

ABC support
oxygen
fluids

81
Q

pathophysiology of acute heamolytic trasnfusion reaction

A

red blood cell destruction by IgM-type antibodies.

82
Q

do reticulocytes have a nucleus

A

no only RNA not DNA

83
Q

what conditions cause MAHA

A

DIC
HUS
TTP

84
Q

what antibodies are involves in RBC destruction in paroxysmal cold haemoglobinuria and paroxysmal nocturnal haemoglobinuria

A

IgG

85
Q

examples of immune mediated intravasculaar haemlysis

A

cold haemoglobinuria

86
Q

what type of haemolysis is osmotic lysis

A

intravascular - hypotonic Iv solution causes RBCs to burst

87
Q

what is produced in liver if high iron stroes to down regulate absorption

A

hepcidin- also produced in anaemia of chronic disease

88
Q

primary vs secondary haemostasis

A

primary - platelet plug
secondary - coagulation cascade

89
Q

in primary haemostasis endothelial damaage causes exposure of what factors

A

collage and VW factor

90
Q

Pt and APTT what one is intrinsic pathway and what one is extrinsic

A

intrinsic - PT
extrinsic - PATT

91
Q

what would cause multiple clotting factor deficiency

A

DIC And liver failure

92
Q

vitamin k dependent clotting factors

A

2,7,9 and 10

93
Q

what do warfarin adn heparin affect with regards to PT and APTT

A

warfarin - PT time
heparin - APTT

94
Q

broad cateogries of casues of failure of platelet plug and what they include

A

VASCULAR - collagen deficiency eg marfans
vasculitis- HSP
scurvy - vit c

PLATELETS - thrombocytopenia
hereditary vs acquires
eg dic, ITP (autoimmune), hypersepnism

VW deficiency

95
Q

casues of failure of secondary haemostasis - fibrin clot

A

clottin gfactor deficiency

multiple- DIC , liver disease
single - haemophilia

96
Q

how might pbstructive jaundice casue clotting factor deficiency

A

bile needed to absorb vit k

97
Q

treatment of ITP in adults

A

steroids

98
Q

inheritance pattern of haemophilia

A

x-linked recessive

99
Q

difference between haemophilia A and B

A

A - VIII deficiency
B - IX deficiency (christmas disease)

100
Q

PT and APTT in haemophilia

A

PT - norm
APTT - prolonged

101
Q

what breaks dwon the fibrin clot

A

plasmin

102
Q

role of tpa in body

A

tissue plasminogen actovator converts plasminogen to plasmin - this breaks dwon fibrin clot

103
Q

what are the naturally occuring anti-coagulatns

A

antithrombin,
protein C and S

104
Q

examples of inherited thrombophilias

A

factor V leiden
protein s deficiency
preotein c deficiency
anti thrombin deficicency
prothrombin 20210 mutation

105
Q

example of an acquired thrombophili

A

anti-phospholipid syndrome

106
Q

features of anti phospholipid

A

arterial and venous thrombosis
fetal loss
mild thrombocytopenia

107
Q

what is seen with Pt and APTT in anti-phospholipid

A

normal PT
prolonged APTT

108
Q

antibodies seen in anti-phospholipid

A

anticardiolipin antibodies
anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
lupus anticoagulant

109
Q

management of anti-phospholipid

A

prophylaxis - asprin
after first event - warfarin

110
Q

MOA of heparin

A

potentiate anti thrombin

111
Q

diff between unfractioned and LMWH MOA’s

A

unfraction- anti-thrombin binds with thrombin
LMWH - anti thrombin binds with factor Xa

112
Q

what form of heparin requires monitoring adn how

A

unfractionated via APTT

113
Q

heparin reversal agent

A

protamine sulphate

114
Q

side effects of heparin

A

osteoporosis
trombocytopenia
bleeding
hyperkalaemia

115
Q

side effects of heparin induced thrombocytopenia

A

pro-thrombotic

116
Q

what juice is avoided with warfarin

A

cranberry

117
Q

why is heparin used alongside warfarin in the first 5 days

A

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
Q

what is required for vitamin K absorption

A

bile

119
Q

why are newborns given vitamin K

A

to avoid haemorrhagic disease of the newborn

120
Q

lifespan of plateele

A

7-10 days