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BCR 1 > Pathology - Anaemias : Diagnosis and Classification > Flashcards

Pathology - Anaemias : Diagnosis and Classification Flashcards

1
Q

which of these is the most common cause of lack of oxygen to the tissues?

a.issues with haem synthesis
b.haemoglobinopathy
c.reduced po2
d.changes to O2 affinity

A

a.issues with haem synthesis

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2
Q

what is anaemia?

A

condition in which the number of red blood cells or their oxygen carrying is insufficient to meet physiologic needs

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3
Q

normal haemoglobin levels in a woman?

a.120-155 g/l
b.130-165 g/l
c. 115-155g/l

A

a.120-155 g/l

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4
Q

normal haemoglobin levels in a man?

a.120-155 g/l
b.130-165 g/l
c. 115-155g/l

A

b.130-165 g/l

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5
Q

normal haemoglobin levels in a newborn?

a.120-155 g/l
b.130-165 g/l
c. 115-155g/l
d.149-237g/l

A

d.149-237 g/l

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6
Q

normal haemoglobin levels in a child up to 12 yrs?

a.120-155 g/l
b.130-165 g/l
c. 115-155g/l
d.149-237 g/l
e.115-155 g/l

A

e.115-155 g/l

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7
Q

which of these is not a physical adaptation to anaemia?

a.producing more rbc
b. changing dynamic properties of o2 dissociation
c.increase volumes of inspired o2
d.increased cardiac output
e.increased respiratory rate

A

e.increased respiratory rate

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8
Q

along with the elderly and very young who is most susceptible to anaemia?

a.those with lung/heart disease

b.obese people

c.people who smoke

d. people with hypertension

A

a.those with lung/heart disease

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9
Q

increased respiratory rate, confusion, pallor, increased heart rate, feeling short of breath, light headed and which other symptom is typical of anaemia?

a.paroxysmal nocturnal dypsnoea

b.orthopnoea

c. palpatations and chest pain

d. finger clubbing

e.nail pittting

A

c. palpatations and chest pain

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10
Q

what are the 3 broad causes of anaemia?

A

failure of production
destruction of red cells
loss of red cells

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11
Q

which of these factors does not influence production of sufficient numbers of functional red cells?

a.haem synthesis
b. haemolysis
c.synthesis of globin chains
d.effective erythropoesis
e.functional bone marrow

A

b. haemolysis

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12
Q

a complex metabolic pathway resulting in the incorporation of which ion results in the formation of haem?

a.Fe3+
b.Fe+
c.Fe2+
d.Fe

A

c.Fe2+

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13
Q

dietary insufficiency and what else can cause reduced absorbption of iron?

a.lyme disease
b.coeliac disease
c. reduced Fe ccontaining enzymes
d.liver failure

A

b.coeliac disease

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14
Q

where is iron absorbed?

a.across the GI epithelial cells

b.across the respiratory epithelial cells

c. across the distal convuluted tubule

d.across the loop of Henle

A

a.across the GI epithelial cells

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15
Q

What is the effect of free intracellular iron?

a.increases erythropoesis

b. decreases erythropoesis

c. toxic to cells

d.causes cell division

A

c.toxic to cells

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16
Q

true or false most iron is found as non haem iron

A

false

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17
Q

which protein is responsible for packaging iron?

a.transferrin
b.ferritin
c.globulin
d.albumin
e.fibrinogen

A

b.ferritin

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18
Q

levels of which protein correspond to iron stores?

a.transferrin
b.ferritin
c.globulin
d.albumin
e.fibrinogen

A

b.ferritin

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19
Q

What can cause ferritin levels to rise despite iron defficiency being present?

a.infection, inflammation, liver disease

b.vomiting

c.acute bledding

d.increased intracellular iron

A

a.infection, inflammation, liver disease

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20
Q

Transferrin levels are… with iron defficiency?

a.increased
b.decreased
c.the same

A

a.increased

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21
Q

ferritin levels are …. in defficiency?

a.increased
b.decreased
c.unchanged

A

b.decreased

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22
Q

which test for iron has significant variation throughout the day and with acute illness?

a.ferritin
b.transferrin
c.transferrin saturation index
d.serum iron

A

d.serum iron

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23
Q

what ratio does transferrin saturation index measure?

a.serum iron/TIBC
b. TIBC/serum iron
c.Ferritin/transferrin
d.transferrin/ferritin

A

a.serum iron/TIBC

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24
Q

What happens to the transferrin saturation index in iron defficiency anaemia?

a.increase
b.unchanged
c.decrease

A

c.decrease

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25
Q

what is the appearance of the red blood cells in iron defficiency anaemia?

a, hyperchromic, macrocytic

b.hypochromic, microcytic

c.normoctic, normochromic

A

b.hypochromic, microcytic

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26
Q

At which point in the successful delivery of oxygen does iron defficiency anaemia pathophysiology occur?

a.synthesus of haem

b.synthesis of globin chains

c.synthesis of red blood cells

A

a.synthesus of haem

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27
Q

what two systems should be considered when investigating the cuase of anaemia ?

a.gynae and resp

b.gI and cardiovascular

c.GI and gynae

d.cardiovascualr and resp

A

c.GI and gynae

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28
Q

Which of these does not cause iron defficiency?

a.chronic blood loss
b.malabsorption - coeliac disease
c. dietary insufficiency
d.thalassemia

A

d.thalassemia

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29
Q

alpha and beta thalassaemia are examples of which type of haemoglobinopathies based in the synthesis of globin?

a.qualitative
b.quantitative

A

b.quantitative

reduced production of functioning globin chains

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30
Q

sickle cell disease is an example of which type of haemoglobinopathy?

a.qualitative
b.quantitative

A

a.qualitative

change in Hb structure or function

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31
Q

What is the effect of B0 type mutations?

a.completely inhibit the synthesis of a globin

b. partly inhibit the synthesis of a globin

c.completely inhibit the synthesis of b globin

d.partly inhibit the synthesis of b globin

A

c.completely inhibit the synthesis of b globin

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32
Q

What is the effect of B+ type mutations?

a.completely inhibit the synthesis of a globin

b. partly inhibit the synthesis of a globin

c.completely inhibit the synthesis of b globin

d.partly inhibit the synthesis of b globin

A

d.partly inhibit the synthesis of b globin

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33
Q

How does B thalassaemia lead to anaemia?

a.haem cannot be made so lack of haemoglobin available to carry o2

b.failure of B globin production means there is an imbalance between a and b chains, a chains are unstable and form intracellular inclusions which interefere with the cell membrane, this causes intermedullary destruction of red cell precursors and a shortened life span of red cells

A

b.failure of B globin production means there is an imbalance between a and b chains, a chains are unstable and form intracellular inclusions which interefere with the cell membrane, this causes intermedullary destruction of red cell precursors and a shortened life span of red cells

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34
Q

at which point in sufficient oxygen delivery does beta thallasemia acts to cause anaemia?

a.circulation
b.production of haem
c.production of globin

A

c.production of globin

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35
Q

which chain is highly unstable when free and can form intracellular inclusions which interfere with the cell membrane causing intermedullary destruction of red cell precursors

a. a globin chains
b. b globin chains
c.haem

A

a.a globin chains

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36
Q

which of these is the correct genotypes for beta thalassemia major ?

a.B0 B0 / B+B0
b.B+ B0 / B+ B+
c.B0 B / B+ B

A

a.B0 B0 / B+B0

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37
Q

which of these is the correct genotypes for beta thalassemia intermedia ?

a.B0 B0 / B+B0
b.B+ B0 / B+ B+
c.B0 B / B+ B

A

b.B+ B0 / B+ B+

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38
Q

which of these is the correct genotypes for beta thalassemia minor ?

a.B0 B0 / B+B0
b.B+ B0 / B+ B+
c.B0 B / B+ B

A

c.B0 B / B+ B

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39
Q

A patient with anaemia undergoes a genetic test to investigate the cause. the results show the genotype B0 B / B+ B what type of thalassemia does this represent ?

a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

a.beta thalassemia minor

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40
Q

A patient with anaemia undergoes a genetic test to investigate the cause. the results show the genotype B0 B+ / B+ B+ what type of thalassemia does this represent ?

a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

b.beta thalassemia intermedia

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41
Q

A patient with anaemia undergoes a genetic test to investigate the cause. the results show the genotype B0 B+0 / B+ B0 what type of thalassemia does this represent ?

a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

c. beta thalassemia major

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42
Q

Which thalassaemia renders a patient transfusion dependent?
a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

c. beta thalassemia major

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43
Q

Which thalassaemia renders a patient transfusion dependent later in life only ?
a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

b.beta thalassemia intermedia

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44
Q

When does beta thalassaemia major onset?

a.1 year
b.2 years
c.3 months
d.6 months

A

d.6 months

as Hbf levels drop

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45
Q

severe profound anaemia, hepatosplenomegaly, skeletal abnormalities,iron overload symptoms secondary to transfusion and high output heart failure are associated with beta thalassemia?

a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

c. beta thalassemia major

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46
Q

mild anaemia with marked microcytosis which may be mistaken for Fe defficiency are associated with beta thalassemia?

a.beta thalassemia minor
b.beta thalassemia intermedia
c. beta thalassemia major

A

a.beta thalassemia minor

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47
Q

what do a0 type mutations do?

a.completely inhibit synthesis of the ⍺globin

b.partially inhibit synthesis of the ⍺globin

A

a. a0 type mutations completely inhibit synthesis of the ⍺globin

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48
Q

what do a+ type mutations do?

a.completely inhibit synthesis of the ⍺globin

b.partially inhibit synthesis of the ⍺globin

A

b.partially inhibit synthesis of the ⍺globin

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49
Q

the genotype a0 a results in which condition?

a. Hb Barts
b. microcytic hypochromic
c.carrier
d.Hb H

A

b. microcytic hypochromic anaemia usually mild

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50
Q

patient has b. microcytic hypochromic anaemia usually mild which a thalassemia genotype is most likely?

a.ao a
b. a0 a0
c. a+ a
d. Hb H

A

a.ao a

51
Q

patient has Hb Barts which a thalassemia genotype is most likely?

a.ao a
b. a0 a0
c. a+ a
d. Hb H

A

b. a0 a0 incompatible with post uterine life

52
Q

patient is unaffected but is a carrier for a thallassaemia which a thalassemia genotype is most likely?

a.ao a
b. a0 a0
c. a+ a
d. Hb H

A

c. a+ a

53
Q

patient has thalassemia intermedia phenotype which a thalassemia genotype is most likely?

a.ao a
b. a0 a0
c. a+ a
d. Hb H

A

d. Hb H

54
Q

which chromosome does the sickle cell mutation occur on?

a.6
b.10
c.11
d.7

A

c.11

55
Q

which gene does the sickle cell mutation occur?

a.6
b.5
c.7
d.8

A

a.6

56
Q

Which two bases swap places in sickle cell disease?

a.C and G
b.A and T
c,G and T
d. A and C

A

b.A and T

57
Q

Which chromosome is the gene for alpha chains found on?

a.11
b.6
c.3
d.14

A

b.6

58
Q

Patient has genotype HbSS what does this indicate?

a.sickle cell trait
b.sickle cell disease
c.sickle cell anaemia

A

b.sickle cell disease

59
Q

Patient has genotype HbAS what does this indicate?

a.sickle cell trait
b.sickle cell disease
c.sickle cell anaemia

A

a.sickle cell trait

60
Q

What is the phenotype of the genotype HbAS?

a.anaemic
b.unaffected
c.sickle cell disease

A

b.unaffected

61
Q

What is sickle cell trait against?

a.Strep pneumonae
b. staph aureus
c. p. falciparum
d. anaemia

A

c. p. falciparum

62
Q

infection, hypoxia, dehydration and what else can trigger the polymerisation of HbS to rope like fibres and cause sickling?

a.alkalosis
b.acidosis

A

b.acidosis

63
Q

Other than cells adhering to the endothelium of the microvasculature how else does sickle cell lead to vaso occlusion?

a.release of free iron and intravascular NO depletion

a.release of free iron and intravascular NO increase

a.release of free haem and intravascular NO depletion

a.release of free globin and intravascular NO depletion

A

a.release of free haem and intravascular NO depletion

64
Q

What type of anaemia is caused by the shortened red cel lifespan in sickle cell disease?

a.haemolytic
b.iron defficient

A

a.haemolytic

65
Q

Painful Crisis
Acute Chest Syndrome
Priapism
Stroke
Acute Anaemia
Aplastic Crisis
Multi-organ Failure
Acute Cholecystitis

are acute complications of what condition?

a.small vessel obstruction in sickle cell disease

b. beta thalassaemia major

c.Barts Hb disease

A

a.small vessel obstruction in sickle cell disease

66
Q

Chronic Complications
Nephropathy
Chronic Pain
Pulmonary Hypertension
Retinopathy
Neurological impairment
Hyposplenism – Infection and Immunodeficiency

are chronic complications in which condition?

a.small vessel obstruction in sickle cell disease

b. beta thalassaemia major

c.Barts Hb disease

A

a.small vessel obstruction in sickle cell disease

67
Q

where are the numbers of reticulocytes high?

a.bone marrow
b.peripheral blood

A

a.bone marrow

68
Q

low number of reticulovytes indicates what?

a.low level of erythropoesis
b.high level of erythropoesis

A

a. low level of erythropoesis

69
Q

high number of reticulovytes indicates what?

a.low level of erythropoesis
b.high level of erythropoesis

A

b.high level of erythropoesis

70
Q

what does number of reticulocytes represent?

a.haemolysis
b. bledding
c. erythropoesis
d.haemoglobin production

A

c. erythropoesis

71
Q

blood film shows rbc with polychromasia what does this indicate?

a.iron defficiency anaemia
b.B12/folate defficiency anaemia
c.high transferrin
d.high retiulocytes

A

d.high retiulocytes

due to retained ribosomal rna

72
Q

Folate and b12 HAVE A KEY ROLE in the formation of..

a.guanine
b.pyrimidines
c.purines
d.purines and pyrimidines

A

d.purines and pyrimidines

73
Q

true or false humans cannot synthesise B12 or folate so rely on dietary intake?

A

true

74
Q

what substance is produced by the gastric parietal cells and is critial to B12 absorption?

a. intrinsic factor
b. extrinsic factor
c.epithelial factor
d.endothelial factor

A

a. intrinsic factor

75
Q

where does B12 absorption occur?

a.spleen
b.distal convuluted tubule
c. proximal convuluted tubuke
d.distal small intestine
e.distal large intestine

A

d.distal small intestine

76
Q

increased cardiac output, increased resp rate, optimisation of Hb o2 binding and what else happens when there is an increase o2 demand?

a.increased Hb from increased circulating Hb

b. increased WBC

c.increased haemolysis

A

a.increased Hb from increased circulating Hb

77
Q

in hypoxic conditions what happens to HIF1a?

a.breaks down
b.released
c.stabilised and binds to hypoxia response element
d. destabilised and binds to hypoxia response element

A

c.stabilised and binds to hypoxia response element

78
Q

where does stabilised HIF1a bind?

a.6th gene on 11th chromonsome

b. 3’ element of the EPO gene

c.6th chromosome

A

b. 3’ element of the EPO gene

79
Q

What does binding of stabilised HIF1a at the 3’ element of the EPO gene cause?

a.decreased EPO gene translation and transcription

a.increased EPO gene translation and transcription

A

a.increased EPO gene translation and transcription

80
Q

where is EPO produced?

a.spleen
b.bone marrow
c.gastric parietal cells
d.interstitial cells of kideny

A

d.interstitial cells of kideny

81
Q

What would you expect from a mutation causing constitutive
activation of HIF?

A

This would stimulate EPO production by binding to the hypoxia response
element of the gene. Increased EPO production would cause increased red
blood cell production Polycythaemia

82
Q

What would you expect to happen to erythropoietin production and
red blood cell production in chronic kidney disease?

A

A Failing kidney will fail to produce sufficient EPO. Therefore erythropoiesis is
suppressed and this results in anaemia. Often patients require EPO injections to
treat anaemia in CKD

83
Q

How does inflammation /malignancy lead to failed RBC production?

a.decreased Hb production

b.increased haemolysis

c.relative decrease in EPO so erythropoesis and increased hepcidin altering iron homeostasis

A

c.relative decrease in EPO so erythropoesis and increased hepcidin altering iron homeostasis

84
Q

relative decrease in EPO so erythropoesis and increased hepcidin altering iron homeostasis resulting in failed rbc production is caused by…

a. a thalassaemia
b. b thalassaemia
c. iron defficiency
d.malignancy/inflammation

A

d.malignancy/inflammation

85
Q

the failure of erythropoiesis occurring in isolation from the
rest of haematopoiesis. It is marked by severe reticulocytopenia
describes what condition?

a. a thalassaemia
b. b thalassaemia
c. iron defficiency
d.malignancy/inflammation
e.red cell aplasia

A

e.red cell aplasia

86
Q

red cell aplasia leads to?

a.haemolysis
b.reticulocytopenia
c.increased RBCs
d. decreased RBCs

A

b.reticulocytopenia

87
Q

Diamond blackfan anaemia is caused by red cell aplasia. how is it acquired?

a.congenital
b.acquired

A

a.congenital

88
Q

what type of anaemia is caused by a gene mutation affecting ribosome function that results in a differentiation blockin the eary stages of red cell development?

a.iron
b.haemolytic
c.diamond blackfan

A

c.diamond blackfan

89
Q

diamond blackfan anemia is a congentital anaemia caused by a failure of erythropoesis. what dos the genetic mutation effect?

a.haem production
b.globin production
c.ribosome function

A

c.ribosome function

90
Q

infection with what can cause a transient block in erythropoesis?

a.staph aureus
b.strep pneum
c. e coli
b. parovirus
b19
d.covid 19

A

b. parovirus
b19

91
Q

Who is at most significant risk of complications following aquired red cell aplasia?

a.patients with sickle cell, haemolytic anaemia

b.patients with hypertension

c.obese patients

A

a.patients with sickle cell, haemolytic anaemia

little red cell reserve and high demand for production of cells so stand still v dangerous

92
Q

Drugs – eg previous cases of antibodies to recombinant EPO injections have
been implicated
• Autoimmunity – eg SLE, RA etc
• Malignancy – lymphomproliferative malignancy, other solid tumours

can all cause what ?

a.iron defficiency
b.haemolytic anaemia
c.red cell aplasia

A

c.rd cell aplasia

93
Q

at which stage of production does red cell aplasia have its affect?

a.failure to produce haem
b.failure to produce chains
c.failure of erythropoesis
d.non functioning bone marrow

A

c.failure of erythropoesis

94
Q

Haematemesis
• Malaena / PR bleeding
• Raised Urea

are examples of what type of bleeding?

a.acute
b.GI
c.gynae

A

b. GI

95
Q

Heavy
Menstruation
• Post Menopausal
bleeding

are examples of what type of bleeding?

a.acute
b.GI
c.gynae

A

c.gynae

96
Q

what does haemolytic anaemia refer to?

A

describe any
process in which there is increased
breakdown or destruction of mature red
blood cells resulting in a shortened life
span and need for increased
erythropoiesis to compensate.

97
Q

when does haemolytic anaemia become clinically apparent?

a. when production> breakdown

b. when Breakdown > Production

A

b. when Breakdown > Production

98
Q

true or false haemolysis can be both intravascular and extravascular

A

true

99
Q

• Anaemia (can be mild severe)
• Raised Bilirubin
• Raised LDH
• Decreased Haptoglobin
• Increased Reticulocytes
• Spherocytes, Schistocytes (fragments)
• Positive Coombes Test

indicate which type of anaemia`?

a.diamond blackfan
b.haemolytic
c. iron defficiency

A

b.haemolytic

100
Q

where in the body is unconjugated billirubin converted to conjugated billirubin by UDP glucuronosyltransferase ?

a.kidney
b.liver
c.spleen
d.bone marrow

A

b.liver

101
Q

what enzyme is responsible for conversion of unconjugated billirubin to conjugated billirubin?

a.heme oxygenase
b.biverdin reductase
c.UDP glucoronosyltransferase

A

c.UDP glucoronosyltransferase

102
Q

what enzyme is responsible for release of iron for recycling?

a.heme oxygenase
b.biverdin reductase
c.UDP glucoronosyltransferase

A

a.heme oxygenase

103
Q

what enzyme is responsible for conversion of bilverdin to bilirubin?

a.heme oxygenase
b.bilverdin reductase
c.UDP glucoronosyltransferase

A

b.bilverdin reductase

104
Q

how is conjugated bilirubin excreted?

a.urine
b.sweat
c.bile

A

c.bile

105
Q

over production of billirubin and and failure to conjugate bilirubin leads to?

a.hypobilirubinaemia
b.hyperbillirubinaemia

A

b.hyperbillirubinaemia

106
Q

Wjat does enhanced haemolysis lead to?

a.overproduction of billirubin

b.failure to conjugate billirubin

A

a.overproduction of billirubin

107
Q

What effect do Gilberts syndrome, Crigler-Najar syndrome and liver failure have on billirubin?

a.overproduction of billirubin
c.failure to conjugate billirubin
d.increased conjugation of billirubin
e.under production of billirubin

A

c.failure to conjugate billirubin

108
Q

what condition i described as a benign condition, characterised by isolated high bilirubin and often precipitated by infection or trauma?

a.Crigler Najar syndrome
b.liver failure
c. Hb H
d.Gilberts syndrome
e.Marfans syndrome

A

d.Gilberts syndrome

109
Q

which of these factors does not precipitate Gilberts syndrome?

a. trauma
b.infection
c.vomiting

A

c.vomiting

110
Q

low haptoglobin levels are present in which type of haemolysis?

a.intravascular
b.acute
c.chronic
d.extra vascular

A

a.intravascular

111
Q

what does Hb combine with intravascurlarly prior to breakdown?

a.heme oxygenase
b.bilverdin reductase
c.UDP glucoronosyltransferase
d.haptoglobin

A

d.haptoglobin

112
Q

What cytoplasmic enzyme is a good marker of cell turnover and is released when red cells are broken down?

a.heme oxygenase
b.bilverdin reductase
c.UDP glucoronosyltransferase
d.Lactate dehydrogenase

A

d.Lactate dehydrogenase

113
Q

lactate dehydrogenase is… in haemolytic anaemia?

a.low
b.high

A

b.high

114
Q

which of these does not cause raised LDH?

a.haemolytic anaemia
b.iron defficiency anaemia
c.malignancy (high grade lymphomas)
d. Tissue damage eg MI/liver failure

A

b.iron defficiency anaemia

115
Q

what is coombes reganet made up of?

a.Antibodies to RBC antigens
b.antihuman antibodies

A

b.antihuman antibodies

116
Q

what is a positive coombes result?

a.clear serum
b.cloudy serum
c.red agglutination

A

c.red agglutination

117
Q

what does a positive coombes result indicate?

a.iron defficiency anaemia
c.haemolytic anaemia
d. immune mediated haemolytic anaemia

A

d. immune mediated haemolytic anaemia

118
Q

which of these drugs does not cause acquired haemolytic anaemia ?

a.cephalosporins
b.NSAIDS
c.penicillin
d.corticosteroids
e.nitrofurantoin/dapsone

A

d.corticosteroids

119
Q

Infection by what can cause acquired haemolytic anaemia
a. e coli
b.malaria
c.hep B

A

b.malaria

120
Q

defficiency of which enzyme can cause congenital haemolytic anaemia?

a.heme oxygenase
b.bilverdin reductase
c.UDP glucoronosyltransferase
d.Lactate dehydrogenase
e.G6PD

A

e.G6PD

121
Q

blood film shows spehrocytes and scistocytes what type of anaemia most likley?

a.iron defficiency anaemia
c.haemolytic anaemia
d. immune mediated haemolytic anaemia

A

d. immune mediated haemolytic anaemia

122
Q

B12 and/or Folate deficiency
• Alcohol,
• Drugs (HU, EPO, Chemotherapy)
• Haemolysis
• Hypothyroidism
• Bone Marrow Infiltration
• Pregnancy

cause what size rbc?

a.microcytic
b.macrocytic
c.normocytic

A

b.macrocytic

123
Q

• Iron Deficiency
• Thalassemia

cause what appearance of rbc?

a.microcytic
b.macrocytic
c.normocytic

A

a.microcytic

124
Q

Acute Bleeding
• Anaemia Chronic Disease
• Mixed Aetiology

cause what appearance of rbc?

a.microcytic
b.macrocytic
c.normocytic

A

c.normocytic

BCR 1 (60 decks)

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