Anaemia

Question 1

select the most appropriate description of underlying disease mechanism for the following haematological condition:
* acute lymphoblastic leukaemia
a - AI suppression of BM stem cells
b - infiltration of BM
c - cytokine induced suppression of marrow function
d - immune destruction by the spleen
e - infiltration of colonal proliferation of mature cells
f - lack of B12
g - deficiency of iron
h - progressive RBC division due to inability to achieve optimum Hb conc
i - lack of erythropoietin
j - genetic abnormality of Hb genes
k - colonal abnormality of stem cells
l - genetic mutation causing constitutively activated tyrosine kinase mediated clonal proliferation and reduced apoptosis
m - genetic abnormality of RBC membrane proteins
n- infiltration by clonal proliferation of primitive cells

Answer 1

n - infiltration by clonal proliferation of primitive cells

Question 2

select the most appropriate description of underlying disease mechanism for the following haematological condition:
* microcytic hypochromic anaemia (small pale red cells)
a - AI suppression of BM stem cells
b - infiltration of BM
c - cytokine induced suppression of marrow function
d - immune destruction by the spleen
e - infiltration of colonal proliferation of mature cells
f - lack of B12
g - deficiency of iron
h - progressive RBC division due to inability to achieve optimum Hb conc
i - lack of erythropoietin
j - genetic abnormality of Hb genes
k - colonal abnormality of stem cells
l - genetic mutation causing constitutively activated tyrosine kinase mediated clonal proliferation and reduced apoptosis
m - genetic abnormality of RBC membrane proteins
n- infiltration by clonal proliferation of primitive cells

Answer 2

h - progressive RBC division due to inability to achieve optimum Hb conc

Question 3

select the most appropriate description of underlying disease mechanism for the following haematological condition:
* aplastic anaemia
a - AI suppression of BM stem cells
b - infiltration of BM
c - cytokine induced suppression of marrow function
d - immune destruction by the spleen
e - infiltration of colonal proliferation of mature cells
f - lack of B12
g - deficiency of iron
h - progressive RBC division due to inability to achieve optimum Hb conc
i - lack of erythropoietin
j - genetic abnormality of Hb genes
k - colonal abnormality of stem cells
l - genetic mutation causing constitutively activated tyrosine kinase mediated clonal proliferation and reduced apoptosis
m - genetic abnormality of RBC membrane proteins
n- infiltration by clonal proliferation of primitive cells

Answer 3

a - AI suppression of BM stem cells

Question 4

select the most appropriate description of underlying disease mechanism for the following haematological condition:
* chronic myeloid leukaemia
a - AI suppression of BM stem cells
b - infiltration of BM
c - cytokine induced suppression of marrow function
d - immune destruction by the spleen
e - infiltration of colonal proliferation of mature cells
f - lack of B12
g - deficiency of iron
h - progressive RBC division due to inability to achieve optimum Hb conc
i - lack of erythropoietin
j - genetic abnormality of Hb genes
k - colonal abnormality of stem cells
l - genetic mutation causing constitutively activated tyrosine kinase mediated clonal proliferation and reduced apoptosis
m - genetic abnormality of RBC membrane proteins
n- infiltration by clonal proliferation of primitive cells

Answer 4

l - genetic mutation causing constitutively activate tyrosine kinase mediated clonal proliferation and reduced apoptosis

Question 5

select the most appropriate description of the underlying disease mechanism for the following haematological diseases:
* myeloma
a - lack of iron
b - BM infiltration by reed sternberg cells, sclerosis and lymphocytes
c - presence of serum paraprotein
d - presence of philedelphia chromosome
e - clonal abnormality of stem cells giving rise to infiltration of BM by primitive myeloid precursors
f - AI suppression of BM stem cells
g - ineffective utilisation of iron
h - suppression of marrow function systemic cytokine release
i - infiltration of BM be clonal proliferation of plasma cells
j - BM stromal damage
k - clonal proliferation and reduced apoptosis driven by constitutively activated tyrosine kinase
l - reduced erythriod maturation due to lack of epo
m - clonal abnormality of stem cells causing failure of functional development of all marrow cell lines
n - proliferation of mature myeloid precursors

Answer 5

i - infiltration of BM by clonal proliferation of plasma cells

Question 6

select the most appropriate description of the underlying disease mechanism for the following haematological diseases:
* anaemia of renal failure
a - lack of iron
b - BM infiltration by reed sternberg cells, sclerosis and lymphocytes
c - presence of serum paraprotein
d - presence of philedelphia chromosome
e - clonal abnormality of stem cells giving rise to infiltration of BM by primitive myeloid precursors
f - AI suppression of BM stem cells
g - ineffective utilisation of iron
h - suppression of marrow function systemic cytokine release
i - infiltration of BM be clonal proliferation of plasma cells
j - BM stromal damage
k - clonal proliferation and reduced apoptosis driven by constitutively activated tyrosine kinase
l - reduced erythriod maturation due to lack of epo
m - clonal abnormality of stem cells causing failure of functional development of all marrow cell lines
n - proliferation of mature myeloid precursors

Answer 6

l - reduced maturation due to reduced epo

Question 7

select the most appropriate description of the underlying disease mechanism for the following haematological diseases:
* hodgkins disease
a - lack of iron
b - BM infiltration by reed sternberg cells, sclerosis and lymphocytes
c - presence of serum paraprotein
d - presence of philedelphia chromosome
e - clonal abnormality of stem cells giving rise to infiltration of BM by primitive myeloid precursors
f - AI suppression of BM stem cells
g - ineffective utilisation of iron
h - suppression of marrow function systemic cytokine release
i - infiltration of BM be clonal proliferation of plasma cells
j - BM stromal damage
k - clonal proliferation and reduced apoptosis driven by constitutively activated tyrosine kinase
l - reduced erythriod maturation due to lack of epo
m - clonal abnormality of stem cells causing failure of functional development of all marrow cell lines
n - proliferation of mature myeloid precursors

Answer 7

b - BM infiltration by reed sternberg cells, sclerosis and lymphocytes

Question 8

select the most appropriate description of the underlying disease mechanism for the following haematological diseases:
* acute myeloid leukaemia
a - lack of iron
b - BM infiltration by reed sternberg cells, sclerosis and lymphocytes
c - presence of serum paraprotein
d - presence of philedelphia chromosome
e - clonal abnormality of stem cells giving rise to infiltration of BM by primitive myeloid precursors
f - AI suppression of BM stem cells
g - ineffective utilisation of iron
h - suppression of marrow function systemic cytokine release
i - infiltration of BM be clonal proliferation of plasma cells
j - BM stromal damage
k - clonal proliferation and reduced apoptosis driven by constitutively activated tyrosine kinase
l - reduced erythriod maturation due to lack of epo
m - clonal abnormality of stem cells causing failure of functional development of all marrow cell lines
n - proliferation of mature myeloid precursors

Answer 8

e - clonal abnormality of stem cells giving rise to infiltration of bone marrow by primitive myeloid precursors

Question 9

select the most appropriate description of the underlying disease mechanism for the following haematological diseases:
* myelodysplasia
a - lack of iron
b - BM infiltration by reed sternberg cells, sclerosis and lymphocytes
c - presence of serum paraprotein
d - presence of philedelphia chromosome
e - clonal abnormality of stem cells giving rise to infiltration of BM by primitive myeloid precursors
f - AI suppression of BM stem cells
g - ineffective utilisation of iron
h - suppression of marrow function systemic cytokine release
i - infiltration of BM be clonal proliferation of plasma cells
j - BM stromal damage
k - clonal proliferation and reduced apoptosis driven by constitutively activated tyrosine kinase
l - reduced erythriod maturation due to lack of epo
m - clonal abnormality of stem cells causing failure of functional development of all marrow cell lines
n - proliferation of mature myeloid precursors

Answer 9

m - clonal abnormality of stem cells causing failure of functional development of all marrow cell lines

Question 10

select the most appropriate disease mechanism for the clinical condition:
* idiopathic thrombocytopenia
a - inherited abnormality of red cell enzyme function
b - clonal proliferation of platelet precursors (megakaryocytes) in the BM
c - BM infiltration by primitive myeloid precursors
d - immune cell suppression of marrow stem cells
e - constitutively activated tyrosine kinase
f - failure of b12 absorption
g - genetic mutation of Hb genes
h - AB mediated immune destruction of red cells by spleen
i - genetic mutation in red cell membrane synthesis
j - drug induced haemolysis
k - AB mediated immune destruction of platelets by spleen
l - reduced erythriod maturation due to lack of epo
m - cytokine mediated BM suppression
n - ineffective utilisation of iron

Answer 10

k - AB mediated immune destruction of platelets by spleen

Question 11

select the most appropriate disease mechanism for the clinical condition:
* hereditary spherocytosis
a - inherited abnormality of red cell enzyme function
b - clonal proliferation of platelet precursors (megakaryocytes) in the BM
c - BM infiltration by primitive myeloid precursors
d - immune cell suppression of marrow stem cells
e - constitutively activated tyrosine kinase
f - failure of b12 absorption
g - genetic mutation of Hb genes
h - AB mediated immune destruction of red cells by spleen
i - genetic mutation in red cell membrane synthesis
j - drug induced haemolysis
k - AB mediated immune destruction of platelets by spleen
l - reduced erythriod maturation due to lack of epo
m - cytokine mediated BM suppression
n - ineffective utilisation of iron

Answer 11

i - genetic mutation in red cell membranes

Question 12

select the most appropriate disease mechanism for the clinical condition:
* aplastic anaemia
a - inherited abnormality of red cell enzyme function
b - clonal proliferation of platelet precursors (megakaryocytes) in the BM
c - BM infiltration by primitive myeloid precursors
d - immune cell suppression of marrow stem cells
e - constitutively activated tyrosine kinase
f - failure of b12 absorption
g - genetic mutation of Hb genes
h - AB mediated immune destruction of red cells by spleen
i - genetic mutation in red cell membrane synthesis
j - drug induced haemolysis
k - AB mediated immune destruction of platelets by spleen
l - reduced erythriod maturation due to lack of epo
m - cytokine mediated BM suppression
n - ineffective utilisation of iron

Answer 12

d - immune suppression of marrow stem cells

Question 13

select the most appropriate disease mechanism for the clinical condition:
* anaemia of chronic renal failure
a - inherited abnormality of red cell enzyme function
b - clonal proliferation of platelet precursors (megakaryocytes) in the BM
c - BM infiltration by primitive myeloid precursors
d - immune cell suppression of marrow stem cells
e - constitutively activated tyrosine kinase
f - failure of b12 absorption
g - genetic mutation of Hb genes
h - AB mediated immune destruction of red cells by spleen
i - genetic mutation in red cell membrane synthesis
j - drug induced haemolysis
k - AB mediated immune destruction of platelets by spleen
l - reduced erythriod maturation due to lack of epo
m - cytokine mediated BM suppression
n - ineffective utilisation of iron

Answer 13

l - reduced epo

Question 14

select the most appropriate disease mechanism for the clinical condition:
* thalassaemia
a - inherited abnormality of red cell enzyme function
b - clonal proliferation of platelet precursors (megakaryocytes) in the BM
c - BM infiltration by primitive myeloid precursors
d - immune cell suppression of marrow stem cells
e - constitutively activated tyrosine kinase
f - failure of b12 absorption
g - genetic mutation of Hb genes
h - AB mediated immune destruction of red cells by spleen
i - genetic mutation in red cell membrane synthesis
j - drug induced haemolysis
k - AB mediated immune destruction of platelets by spleen
l - reduced erythriod maturation due to lack of epo
m - cytokine mediated BM suppression
n - ineffective utilisation of iron

Answer 14

g - genetic mutation of Hb genes

Question 15

for the following examples of bleeding, select the most appropriate disease mechanism or pathology
* mucosal bleeding - epistaxis, buccul blood blisters - and peripheral petichial rash
a - aspirin therapy
b - over active platelets
c - haemophilia
d - vWB disease
e - reduced coag factors or function
f - deficiency of vit K
g - high INR secondary to warfarin
h - factor V Leidin deficiency
i - consumption coagulopathy secondary to sepsis (DIC)
j - therapeutic heparin infusion for ACS
k - reduced platelet numbers or fuction
l - antiphospholipid syndrome
m - protein malnutrition
n - eherlers danlos syndrome

Answer 15

k - reduced platelet number or function

Question 16

for the following examples of bleeding, select the most appropriate disease mechanism or pathology
* deep muscle haematoma developing over 24hrs post trauma
a - aspirin therapy
b - over active platelets
c - haemophilia
d - vWB disease
e - reduced coag factors or function
f - deficiency of vit K
g - high INR secondary to warfarin
h - factor V Leidin deficiency
i - consumption coagulopathy secondary to sepsis (DIC)
j - therapeutic heparin infusion for ACS
k - reduced platelet numbers or fuction
l - antiphospholipid syndrome
m - protein malnutrition
n - eherlers danlos syndrome

Answer 16

c - haemophilia

Question 17

for the following examples of bleeding, select the most appropriate disease mechanism or pathology
* generalised oozing from venepuncture sites and wound edges following surgery for ruptured bowel
a - aspirin therapy
b - over active platelets
c - haemophilia
d - vWB disease
e - reduced coag factors or function
f - deficiency of vit K
g - high INR secondary to warfarin
h - factor V Leidin deficiency
i - consumption coagulopathy secondary to sepsis (DIC)
j - therapeutic heparin infusion for ACS
k - reduced platelet numbers or fuction
l - antiphospholipid syndrome
m - protein malnutrition
n - eherlers danlos syndrome

Answer 17

i - consumption of coagulopathy secondary to sepsis (DIC)

Question 18

for the following examples of bleeding, select the most appropriate disease mechanism or pathology
* intracerebral bleed in elderly patient with AF
a - aspirin therapy
b - over active platelets
c - haemophilia
d - vWB disease
e - reduced coag factors or function
f - deficiency of vit K
g - high INR secondary to warfarin
h - factor V Leidin deficiency
i - consumption coagulopathy secondary to sepsis (DIC)
j - therapeutic heparin infusion for ACS
k - reduced platelet numbers or fuction
l - antiphospholipid syndrome
m - protein malnutrition
n - eherlers danlos syndrome

Answer 18

g - high INR secondary to warfarin therapy

Question 19

for the following examples of bleeding, select the most appropriate disease mechanism or pathology
* bruising and severe GI bleed in a jaundiced patient 
a - aspirin therapy
b - over active platelets
c - haemophilia
d - vWB disease
e - reduced coag factors or function
f - deficiency of vit K
g - high INR secondary to warfarin
h - factor V Leidin deficiency
i - consumption coagulopathy secondary to sepsis (DIC)
j - therapeutic heparin infusion for ACS
k - reduced platelet numbers or fuction
l - antiphospholipid syndrome
m - protein malnutrition
n - eherlers danlos syndrome

Answer 19

e - reduced coagulation factors or function

Question 20

select the most appropriate description of underlying disease mechanism for the following haematological condition:
* anaemia of chronic disease
a - AI suppression of BM stem cells
b - infiltration of BM
c - cytokine induced suppression of marrow function
d - immune destruction by the spleen
e - infiltration of colonal proliferation of mature cells
f - lack of B12
g - deficiency of iron
h - progressive RBC division due to inability to achieve optimum Hb conc
i - lack of erythropoietin
j - genetic abnormality of Hb genes
k - colonal abnormality of stem cells
l - genetic mutation causing constitutively activated tyrosine kinase mediated clonal proliferation and reduced apoptosis
m - genetic abnormality of RBC membrane proteins
n- infiltration by clonal proliferation of primitive cells

Answer 20

c - cytokine induced suppression of marrow function

Question 21

what is a normal Hb level for M and F?

Answer 21

M

Question 22

what controls RBC differentiation?

Answer 22

EPO

growth factors

Question 23

what controls differentiation of WBCs?

Answer 23

GCSF
GMSF
BM stroma cells

Question 24

what would chronic renal disease do to EPO and RBCs?

Answer 24

dec EPO

normocytic anaemia

Question 25

what would chronic anaemia do to EPO and RBCs?

Answer 25

inc EPO | microcytic anaemia

Question 26

what causes frontal bossing and in which disease would you see it in?

Answer 26

- fatty deposits in BM | - thalassaemia paeds

Question 27

why does chronic disease cause anaemia?

Answer 27

shortens RBC life cycle- cytokines from prolonged inflammation interfere

Question 28

what are the 3 haemantics?

Answer 28

iron vit b12 folate

Question 29

what type of anaemia would you get with iron deficiency?

Answer 29

microcytic hypochromic

Question 30

what type of anaemia would you get with vit b12/folate deficiency?

Answer 30

macrocytic megaloblastic

Question 31

what are the signs of pernicious anaemia?

Answer 31

vitileigo lemon yellow tinge glossitis

Question 32

what happens in pernicious anaemia?

Answer 32

IgG autoABs target parietal cells and IF | dec b12 absorption in distal ileum as no IF

Question 33

what kind of anaemia would you get if you had a problem with one of the 4 proteins in the RBC membrane?

Answer 33

haemolytic anaemia

Question 34

which disease is this - dec synthesis of either alpha of beta globin chains

Answer 34

thalassaemia

Question 35

which disease is this - inheritance of 2 abnormal beta chain genes

Answer 35

sickle cell

Question 36

what type of anaemia is thalassaemia?

Answer 36

microcytic hypochromic

Question 37

how would you manage thalassaemia?

Answer 37

blood transfusions | iron chelators

Question 38

how would you manage sickle cell anaemia?

Answer 38

analgesia fluids ABx

Question 39

define haemolysis?

Answer 39

any process that shortens the life cycle of the RBC

Question 40

what will be high in haemolytic anaemia of FBC?

Answer 40

reticulocytes

Question 41

which molecule helps offload O2 into tissues?

Answer 41

2,3DPG

Question 42

what is the difference between HbF and Hb adult?

Answer 42

HbF = 2alpha and 2gamma | Hb adult = 2alpha 2beta

Question 43

what would shift the O2 dissociation curve left, increasing its affinity for O2?

Answer 43

dec CO2 and 2,3DPG | inc pH

Question 44

what would shift the O2 dissociation curve to the right, decreasing the affinity for O2?

Answer 44

inc CO2 and 2,3DPG | dec pH

Question 45

which enzyme breaks down RBCs?

Answer 45

lactic acid dehydrogenase

Question 46

what might the pulse feel like in someone with anaemia?

Answer 46

bounding

Question 47

WRT anaemia - what would cause glossitis?

Answer 47

B12/folate def

Question 48

WRT anaemia - what would cause angular stomitis?

Answer 48

B12/folate def

Question 49

WRT anaemia - what would cause dysphagia?

Answer 49

B12 deficiency

Question 50

WRT anaemia - what would cause kiolonychia?

Answer 50

iron deficiency

Question 51

if the RBC distribution width was high, what would this indicate?

Answer 51

inc reticulocytes - most common cause

Question 52

which type of anaemia is not caused by a problem with DNA synthesis?

Answer 52

microcytic

Question 53

what are some of the possible causes of microcytic anaemia?

Answer 53

iron deficiency thalassaemia sickle cell chronic disease

Question 54

what is the problem in normocytic anaemia?

Answer 54

dec in production of RBCs

Question 55

what could cause normocytic anaemia?

Answer 55

``` chronic disease aplastic anaemia haemolysis pregnancy fluid overload ```

Question 56

what causes RBCs to become macrocytic?

Answer 56

dec life length

Question 57

what could cause macrocytic anaemia?

Answer 57

``` B12/folate def alcohol liver disease hypothyroid hypoxia cytotoxic drugs pregnancy ```

Question 58

what are the 4 categories of causes of haemolysis?

Answer 58

1 - immune 2 - hypersplenism 3 - microangiopathic 4 - infections

Question 59

which microangiopathic disorders could cause haemolysis?

Answer 59

TTP | HUS

Question 60

which test would you use for testing AI haemolysis?

Answer 60

coombe's test

Question 61

who might you see functional hyposplenism in?

Answer 61

HbSS | coeliacs

Question 62

what could cause hydrops fetalis?

Answer 62

2nd baby and no anti-d | thalassaemias

Question 63

what could casue aplastic anaemia?

Answer 63

AI post-viral CTx/RTx

Question 64

when might you see splenomegaly and frontal bossing?

Answer 64

thalassaemias

Question 65

when might you see 'hair on end' on and MRI?

Answer 65

thalassaemias

Question 66

what disease might it be if you see jaundice, pallor and infected LNs?

Answer 66

sickle cell crisis

Question 67

which haemoglobinopathy might you see short fingers in and why?

Answer 67

sickle cell - dactlyitis in infancy

Question 68

which anaemia might you see bony crisis, stroke, tissue infarction and AVN in?

Answer 68

sickle cell

Question 69

WRT FFP - who can have what?

Answer 69

O can have O, A, B or AB A can have A or AB B can have B or AB AB can have AB so the opposite to RBCs

Question 70

WRT blood - who can have what?

Answer 70

O can only have O A can have O, A B can have O and B AB can have O, A, B and AB