Block 4 Revision Sba

2001 SBA RFA 2
Case 6
Miriam Liebling, a 37 year old woman, presents to the GP complaining of tiredness and lethargy.
She has 3 children aged 9, 5 and 4 years. She is not vegetarian and eats a normal Western diet. She
has had menorrhagia for the last year but has been too busy to seek medical help. Her haemoglobin
is measured and the lab phones to say the Hb measures 64 g/L (NR 125-160g/L).
32. Erythropoiesis is stimulated principally by which one of the following?
A) Reduced pulmonary venous oxygen tension
B) Reduced oxygen tension in renal arterial blood
C) Reduced firing of a chemoreceptor in the carotid arteries
D) Reduced secretion of an inhibitory adrenocortical hormone
Answer B
Erythropoietin is secreted principally in response to hypoxia in arterial blood perfusing the kidneys
Author: David Gottlieb, Haematology
Curriculum Reference: 4.01 LT2
33. With respect to erythropoiesis, cellular iron is acquired from circulating:
A) Cytochromes
B) Erythropoietin
C) Ferritin
D) Transferrin
Answer D
Transferrin is the form in which iron is provided to cells. Activation of the transferrin receptor
promotes iron uptake and erythropoiesis.
Author: Arthur Conigrave, Biochemistry
34. Which one of the following would be the best screening test for patients with iron deficiency?
A) Serum iron
B) Transferrin concentration
C) Percentage transferrin saturation with iron
D) Serum ferritin
Answer D
A low serum ferritin is both sensitive and specific for iron deficiency. All other tests are confounded
by recent iron intake or the effects of chronic disease on iron metabolism.
35. Which one of the following is most helpful in indicating a likely cause for the reduced
haemoglobin in a patient with anaemia?
A) The severity of the anaemia
B) The mean corpuscular volume (MCV)
C) The presence of a normal platelet count
D) The patients age
Answer B
None of the other parameters significantly narrow the diagnostic possibilities in patients with
anaemia.
Author: D Gottlieb, Haematology
Curriculum Reference: 4.01 Lecture 3
Case 7
Maria Dimitrious, a 9 month old girl, is brought into your general practice by her mother and
grandmother. They have just immigrated to Australia from Greece. Maria presents with a one month
history of poor feeding, failure to thrive and irritability. On examination her weight is on the 3rd
percentile for age, she is pale, has marked hepatosplenomegaly and a hyperdynamic circulation. A
full blood count reveals the following results:
Indices
Marias Results
Normal range for this age
Hb
*45 g/L
95 140 g/L
MCV
*63 fl
70 85 fl
Hematocrit
*14%
28 45%
WCC
16 x 109/L
5 17 x 109 /L
Platelet Count
409 x 109/L
150 600 x109/L
36. If Maria has thalassemia major as you suspect, her blood film will show:
A) Hypochromic red cells
B) Macrocytic red cells
C) Spherocytic red cells
D) Hyperchromic red cells
Answer A
The blood film in thalassemia major shows hypochromic usually microcytic cells with marked
anisocytosis (variation in size) and poikilocytosis (variation in shape) associated with the presence
of nucleated red blood cells. Spherocytes are not a feature of thalassemia major.
Author: Julie Curtin Dept of Haematology, Childrens Hospital at Westmead.
Curriculum references: 4.01 LT5, 4.02 Lect 1,4
37. Antenatal diagnosis of -thalassemia major is most commonly confirmed by:
A) A full blood count on foetal blood
B) Haemoglobin electrophoresis on foetal blood
C) DNA molecular tests on a chorionic villus sample
D) /globin synthesis ratios on chorionic villus sample
Answer C
Antenatal diagnosis of beta thalassemia major is most commonly confirmed by molecular techniques
(eg RFLP analysis) on a CVS sample.
Curriculum references: 4.02 Lect 4
38. Which of the following investigations should be performed to confirm a suspected diagnosis of
thalassemia major in Maria?
A) Polymerase chain reaction
B) Haemoglobin electrophoresis
C) Restriction fragment length polymorphism analysis

D) Karyotype analysis
Answer B
The diagnosis of thalassemia major is confirmed by haemoglobin electrophoresis. The polymerase
chain reaction and restriction length polymorphism analysis may be used in the process of antenatal
diagnosis but are not required to make the diagnosis of thalassemia major in a patient presenting in
this manner. Karyotype analysis does not provide any useful information in the diagnosis of
thalassemia major.
Curriculum references: 4.02 Lect 1,4
39. In beta thalassemia major there is:
A) Inadequate production of normal alpha chains
B) Normal production of abnormal alpha chains
C) Inadequate production of normal beta chains
D) Normal production of abnormal beta chains
Answer C
In beta thalassemia major there is inadequate production of normal beta globin chains
Case 8
Samuel Irvine, an 18 month old haemophiliac, presents with a right knee haemarthrosis to the
Emergency Department of a district hospital. He has had a previous admission to the same hospital
for the same problem.
40. In children, the clinical finding most suggestive of an inherited coagulation factor defect would
be:
A) Bruises on both shins from an unknown cause
B) Bruises on his chest wall from lying on a toy
C) Sudden onset of petechia
D) Recurrent nose bleeds lasting for 1 minute
Answer B
Bruises on both shins are common in children and do not indicate a bleeding disorder, whereas
bruises on the trunk are uncommon and often indicate a bleeding disorder. Sudden onset of
petechiae is characteristic of ITP. Recurrent nose bleeds of short duration suggest a local problem
eg, bleeding blood vessel.
41. Which of the following screening tests is abnormal in patients with haemophilia A?
A) Platelet count
B) Activated Partial Thromboplastin Time (APTT)
C) Prothrombin Time (PT)
D) Thrombin Time (TT)
Answer B
The deficiency of factor VIII causes prolongation of the APTT

Curriculum references: 4.03 LT4
42. The factor VIII gene encodes a protein of approximately 2,300 amino acids. Given that the gene
spans 186 kilobases of the X chromosome, it is accurate to say that the majority of the DNA in the
factor VIII gene is organized into:
A) Exons
B) Introns
C) Protein encoding regions
D) RNA encoding regions
Answer B
6,900 bases are required to specify a protein of this length. The gene is 20-30 times this size. Exons,
protein-encoding regions and RNA-encoding regions are related terms.
43. Circulating Von Willebrands factor is a protein that influences coagulation by acting to:
A) Degrade factor VIII by proteolysis
B) Convert factor VIII to its active form
C) Stabilize factor VIII in the circulation
D) Cross-link factor VIII to its receptors
Answer C
Von Willebrands factor stabilises Factor 8 in the circulation. As a result, in Von Willebrand's
disease, a secondary deficiency of factor VIII can arise.
44. In haemophilia:
A) The molecular mutation is the same in all patients
B) The family history is negative in over 50% of patients
C) In families with informative RFLP analysis, intragenic markers are more accurate than extragenic
markers
D) The mother of a severe haemophiliac is unlikely to be a carrier if the family history is negative
Answer C
Over 150 mutations have been identified in the Factor VIII gene. Isolated cases representing new
mutations occurring in a non carrier mother or in the grandparent generation are seen in around a
third of patients with haemophilia. A mutation hotspot has been found in 25% of all
haemophiliacs and results in severe disease. Nearly every mother of a sporadic severely affected
patient with an inversion is likely to be a carrier. Because of the large number of mutations causing
haemophilia A, in most cases indirect testing using linked DNA polymorphisms must be employed .
RFLP analysis is informative in most cases, with a 2-6% error rate using extragenic markers and
<1% for informative intragenic markers.
Curriculum references: 4.03 LT6
Case 9
Michelle Yuen, a 26 year old woman, presented to the antenatal clinic at 28 weeks pregnancy. She
had not previously sought antenatal care. She reported that she smokes 5 cigarettes daily and drinks
10-20 g alcohol daily. You noticed that she was pale and proceeded to do a full blood count and
blood film. The results are given in the table below.
Indices
Ms Yuens Results
Normal Range
Hb
*78 g/L
115 165 g/L
MCV
*102 fl
76 96 fl
MCH
30 pg
27 32 pg
WCC
*1.8 x 109/L
4.0 11.0 x 109 /L
Platelet Count
*120 x 109/L
150 500 x109/L
Investigation confirms folate deficiency with a normal B12 level.
45. Which one of the following disorders is never associated with pancytopenia?
A) Folate deficiency
B) Iron deficiency
C) Vitamin B12 deficiency
D) Aplastic anaemia
Answer B
Only iron deficiency is never associated with pancytopenia. Severe megaloblastic anaemia can be
and aplastic anaemia is due to a stem cell disorder which frequently affects all cell lines.
Curriculum Reference: 4.04
46. Which one of the following statements best describes the biochemical abnormality underlying
megaloblastic anaemia?
A) Reduction in levels of folic acid or vitamin B12 impairs the ability of purine analogues to be
converted to pyrimidines
B) Absence of folic acid impairs the conversion of homocysteine to pyrimidines
C) Folic acid and vitaminB12 deficiency combine to inhibit the enzyme dihydrofolate reductase and
pyrimidine synthesis
D) Low levels of folic acid or vitamin B12 impair one-carbon transfer important in the production of
folate polyglutamates and generation of pyrimidines
Answer D
Folic acid and vitamin B12 co-operate in the transfer of a methyl group from methyl
tetrahydrofolate to tetrahydrofolate (during which homocysteine is converted to methionine).
Following this, tetrahydrofolate polyglutamate derivatives act as co-enzymes in the production of
the pyrimidine thymidine.
Curriculum Reference: 4.01 Lecture 4
47. Red cell breakdown (haemolysis) is associated with which one of the following?
A) Reduced level of lactate dehydrogenase
B) Elevated serum haptoglobin levels
C) Erythroid hyperplasia in the spleen
D) Erythroid hyperplasia in the bone marrow

Answer D
Lactate dehydrogenase levels are increased in haemolysis, whereas haptoglobin levels are
decreased.
Erythroid hyperplasia in the bone marrow occurs as a compensatory mechanism to increase red cell
production.
Author: David Gotlieb
Curriculum Reference: 4.04 LT 4
48. A hematocrit of 14% means that, in the sample of blood analysed:
A) 14% of the haemoglobin is in the plasma
B) 14% of the total blood volume is blood plasma
C) 14% of the total blood volume is the red cells
D) 14% of the haemoglobin is in red cells
Answer C
The hematocrit is the percentage of the total blood volume occupied by red cells (or, more
accurately, red and white cells).
Curriculum Reference:4.01, Theme Session B&CS 1 (Laboratory skills - full blood count and
coagulation),4.01, Theme Session B&CS 2
49. Which one of the following changes in the blood is rarely seen during pregnancy?
A) Anaemia
B) Neutrophilia
C) Thrombocytopenia
D) Reduced factor VIII level
Answer D
Levels of plasma coagulation factors including factor VIII frequently rise during and shortly after
pregnancy contributing to the increased incidence of thrombosis observed during this period.
Case 10
Keith Harris, a 73 year old man, is brought to the Emergency Department by ambulance. During the
night he noticed palpitations and shortness of breath which got steadily worse over several hours.
There was no associated chest discomfort although he has had 3 documented myocardial infarcts
over the last 5 years. More chronic problems include ankle swelling and excess alcohol ingestion.
55. The most characteristic pathological finding in a reperfused myocardial infarct, examined three
weeks after the event is:
A) Coagulative necrosis
B) Collagen deposition
C) Lymphocyte infiltration
D) Contraction bands
Answer B
Author: Prof Clive Harper, Pathology
Curriculum Reference: 4.05, Theme Session 1 (Coronary artery disease)

Case 12
Hannah Kovalenko is a 4 year old girl who is noted on routine medical examination to have a heart
murmur. She is referred to a paediatric cardiologist who diagnoses a small ventricular septal defect.
68. Should Hannah be suffering bacterial endocarditis, the most likely causative agent would be:
A) A species of oral streptococci
B) Staphylococcus aureus
C) Coagulase negative staphylococci
D) A Gram-negative (enteric) rod
Answer A
Although almost any organism can cause endocarditis, the majority of cases are caused by a
relatively small range of species. In Hannahs case since she has her own heart valve as opposed to
a prosthetic valve and she is not an i.v. drug user then the most likely causative agent is a species of
oral streptococci e.g., Strep. sanguis
Author: A/Prof. R. Kearney, Infectious diseases
Curriculum reference: 4.03: Lect. 1 Rheumatic valve disease, 4.03: LT.6 Complications of rheumatic
heart disease
2002 RFA2 2002
Case 7
Mr Frederick Worthington is a 49 year old company director. He comes into your surgery because
he
has developed some epigastric discomfort and nausea over the last few weeks. He has also noticed
some shortness of breath and palpitations when walking up hills.
On examination, he looks quite pale, and his pulse is 100. His blood pressure is 175/75. You cannot
palpate an enlarged spleen, and there is no abdominal tenderness.
You order a blood count, which shows:
Indices
Hb
MCV
MCH
MCHC
WCC
Platelet count
Mr Worthington's Results
85 g/L
70 fL
22 pg
314 g/L
5.5 x 109/L
575 x 109/L
Normal Range
130-180 g/L
80-100 fL
27-32
300-350 g/L
4.0-11.0 xl09/L
150-400 x 10y/L
30. Given Mr Worthington's history, examination and findings, the most likely diagnosis is:
A) Aplastic anaemia
B) Pernicious anaemia
C) Iron deficiency anaemia
D) Auto-immune haemolytic anaemia
Answer C
Explanation: The history and physical findings are consistent with anaemia, and there is also a
suggestion of abdominal pathology. The blood film shows microcytosis and hypochromia, typical of
iron deficiency, and the thrombocytosis suggests blood loss is the likely culprit. Pernicious anaemia
is
associated with a macrocytosis. Auto-immune haemolytic is associated with splenomegaly. Aplastic
anaemia is characterised by pancytopenia.
Reference: 4.01, Learning Topic 5 (Clinical and laboratory assessment of anaemia) 4.04,Learning
Topic 1 (Microcytosis)
Curator (for 2002): AlProf David Gottlieb (Department of Haematology, Westmead Hospital)
You ask Mr Worthington some more questions.

31. The question most relevant to your working diagnosis is:
A) Has he noticed that he bruises easily?
B) Does he have a family history of diabetes?
C) Has he recently been taking chloramphenicol?
D) Has he noticed blood in his bowel motions?
Answer D
Explanation: Easy bruising suggests thrombocytopenia, which he does not have. Chloramphenicol
can cause aplastic anaemia, but not iron deficiency. Blood loss from the gastrointestinal tract is a
common cause of iron deficiency anaemia, particularly in the context of epigastric pain and
coexisting
thrombocytosis.
Reference: 4.03, Lecture 1 (Physiology of coagulation -platelet and clotting factors) 4.01, Learning
Topic 3 (Consequences of deficiencies of essential haematinics) 4.01, Lecture 4 (Iron deficiency)
Curator (for 2002):
A/Prof David Gottlieb (Department of Haematology, Westmead Hospital)
32. You go back to the blood film and examine it closely. The most likely finding on the blood film
of this patient is:
A) Hypochromic erythrocytes
B) Macrocytic erythrocytes
C) Spherocytic erythrocytes
D) Fragmented erythrocytes
Answer A
Explanation: The red cells in iron deficiency are hypochromic (low MCH) as well as microcytic
(low
MCV), not macrocytic. Spherocytes are typically found in autoimmune haemolytic anaemia and
hereditary spherocytosis. Fragmented red cells are seen in renal insufficiency, severe burns, and
microangiopathic haemolytic anaemia, not in iron deficiency.
Reference: 4.01, Learning Topic 5 (Clinical and laboratory assessment of anaemia 4.04, Theme
Session B&CS 1 (Interpreting lab tests in anaemia)
Curator (for 2002):
A/Prof David Gottlieb (Department of Haematology, Westmead Hospital)
33. You decide to confirm your diagnosis with additional blood tests. The set of results you would
expect to find is:
A) Low serum iron, high serum transferrin, and low serum ferritin
B) Low serum Bi2 level
C) Low serum iron, low serum transferrin, and normal serum ferritin
D) Reduced haptoglobin levels
Answer A
Explanation: The pattern described in A. is typical of iron deficiency anaemia. Low serum B12 is a
cause of megaloblastic anaemia. Low serum iron, low serum transferrin and normal serum ferritin
are
found in the anaemia of chronic disease. Reduced haptoglobin levels are associated with haemolytic
anaemias.
Reference: 4.01, Lecture 1 (Introduction to anaemia)
4.01, Lecture 3 (Megaloblastic anaemia)
4.01, Learning Topic 5 (Clinical and laboratory assessment of anaemia)
4.05, Learning Topic 7 (Tests of iron status in anaemia)
Curator (for 2002): A/Prof David Gottlieb (Department of Haematology, Westmead Hospital)
34. Transfer of oxygen from blood to tissues is:
A) Increased by 2,3-diphosphoglycerate (2,3-DPG)
B) Decreased by lowering the pH
C) Enhanced by the presence of fetal haemoglobin (HbF) rather than adult haemoglobin
(HbA) in red cells
D) Increased by carbon monoxide
Answer A
Explanation: 2,3-DPG lowers the affinity of haemoglobin for oxygen, thereby facilitating oxygen
delivery to the tissues. Acidosis (lower pH) lowers the affinity of haemoglobin for oxygen, resulting
in
a shift to the right of the haemoglobin oxygen dissociation curve. Fetal haemoglobin has a greater
affinity for oxygen than does adult haemoglobin. Carbon monoxide binds avidly to haemoglobin and
thereby displaces oxygen.
Reference: 4.01, Lecture 2 (Role of haemoglobin in oxygen delivery to tissues)
35. Red cell breakdown (haemolysis) is associated with:
A) Reduced serum bilirubin
B) Elevated serum haptoglobin levels
C) Erythroid hyperplasia in the bone marrow
D) Decreased erythropoietin levels
Answer C
Explanation: Bilirubin is increased in haemolysis, whereas haptoglobin levels are decreased.
Erythroid hyperplasia occurs as a compensatory mechanism to increase red cell production.
Erythropoietin levels are increased in haemolytic anaemias.

Reference: 4.01, Learning Topic 4 (Premature destruction of red blood cells) 4.04, Learning Topic 6
(Shortened red cell survival)
36. In assessing a blood count, a haematocrit of 29% means that in the sample of blood analyzed:
A) 29% of the haemoglobin is in the plasma
B) 29% of the total blood volume is red cells
C) 29% of the haemoglobin is in red cells
D) 29% of the formed elements in blood are red blood cells
Answer B
Reference: 4.02, Theme Session (Interpreting lab tests in anaemia)
Curator: Dr Julie Curtin (New Children's Hospital)
Case 8
Ms Rosa Lipowski, aged 52, comes to see you. She has been unwell for 2 weeks, with bruising,
recurrent epistaxis, and some vaginal spotting. There is no lymphadenopathy or
hepatosplenomegaly.
You arrange a full blood count, and the results are as follows:
Hb 96 g/L (normal range 115-165 g/L)
WCC 2.6 x 109/L (normal range 4.0-11.0 x 109/L) with 60% blasts, some with Auer rods
Platelet count 41 x 109/L (normal range 150-400 x 109/L. A diagnosis of acute myeloblasts
leukaemia is made.
37. The feature of greatest prognostic importance in patients with acute leukaemia is:
A) White cell count at diagnosis
B) Bone marrow cytogenetics
C) Platelet count at diagnosis
D) Physical fitness at diagnosis
Answer B
Explanation: Cytogenetics are now more important than the FAB classification in terms of
prognostic
significance. White cell count at diagnosis is prognostic but less important. The other parameters
have
no significant prognostic value.
Reference: 4.02, Lecture 1 (Leukaemia -pathogenesis and pathophysiology) 4.02, Lecture 2
(Leukaemia clinical patterns) 4.02, Lecture 4 (Leukaemia bench to bedside)
Curator: A/Prof David Gottlieb (Department of Haematology, Westmead Hospital)
38. In acute leukaemia:
A) Blast cells are always present in the blood
B) Blast cells are always present in the bone marrow
C) The white cell count is always high
D) Pancytopenia is always present
Answer B
Explanation: At least 30% of nucleated marrow cells must be blast cells to qualify for a diagnosis of
acute leukaemia. Blast cells may not be found in the peripheral blood. Patients may have normal
blood counts or may be leucopenic.
Reference: 4.02, Learning Topic 5 (Bone marrow failure and stem cell transplant) 4.02, Lecture 1
(Leukaemia -pathogenesis and pathophysiology)
39. The anaemia associated with acute myeloid leukaemia is predominantly due to:
A) Haemolysis
B) Blood loss
C) Renal failure
D) Erythroid hypoplasia
Answer D
Explanation: Cytopenia due to acute myeloid leukaemia is usually due to bone marrow infiltration
with malignant cells, and hypoplasia of normal marrow elements. Haemolysis associated with acute
myeloid leukaemia is rare and, while blood loss associated with thrombocytopenia is common,
anaemia resulting from it is not. Most patients do not present with renal failure.
Reference: 4.02, Learning Topic 2 (Leucocytes and their function) 4.02, Learning Topic 5 (Bone
marrow failure and stem cell transplant) 4.02, Lecture 1 (Leukaemia -pathogenesis and
pathophysiology)
Case 9
Shane Dodds, a 17 year old Year 12 student, recently visited Papua New Guinea in the school
holidays for 3 weeks during which time he spent 3 nights in a coastal village. He took chloroquine
regularly while he was away, but has not taken any antimalarial prophylaxis since his return to
Australia 2 weeks ago. He comes to your general practice surgery now having been unwell for 3
days, experiencing a rigor followed by drenching sweats each night.
40. In malaria:
A) Daily fevers are unusual
B) Recurrences of Plasmodium falciparum are due to failure to eradicate parasites from the
liver
C) A first attack of malaria may occur many months after exposure to infection
D) The diagnosis cannot be made from the microscopic examination of a routinely stained
blood film
Answer C
Explanation: In the initial stages of malaria infection daily fevers are common. Plasmodium vivax
and P. ovale (but not P. falciparum), are associated with persistence in the liver, which requires
eradication with specific therapy after the initial parasitaemia has been cleared. A first attack of
malaria may occur many months after exposure to infection. The diagnosis of malaria can
frequently be made by examining a routinely stained peripheral blood film.
Reference: 4.04, Learning Topic 3 (Fever) 4.04, Learning Topic 4 (Life cycle of malaria parasites)
4.04, Lecture 3 (Diagnosis of malaria) 4.04, Lecture 5 (Common parasitic diseases) Curator: Dr
Julie Curtin (New Children's Hospital)
41. In malaria:
A) Infection may be acquired by a blood transfusion
B) Plasmodium vivax parasites may persist undetected in the circulation for 20 years or more
C) Cerebral malaria is the most common cause of death in Plasmodium vivax infection
A) The highest incidence of malarial infection in infants is in the first month of life
Answer A
Explanation: Malaria can be acquired by blood transfusion from an infected donor. Plasmodium
malariae is the only plasmodial species which may persist in the circulation for decades. Cerebral
malaria is a common cause of death in falciparum, not vivax infections. The new-born infant has
relative protection from malaria through circulating maternal antibodies, but this protection is lost
by
3 to 6 months of age.
Reference: 4.04, Learning Topic 3 (Fever)
4.04, Learning Topic 4 (Life cycle of malaria parasites) 4.04, Lecture 3 (Diagnosis of malaria) 4.04,
Lecture 5 (Common parasitic diseases)
Curator: Dr Julie Curtin (New Children's Hospital
Case 10
Mrs Campbell is a 78 year old grandmother who lives alone, is not eating properly and seems to
have no energy. She has difficulty climbing stairs, and is breathless on minimal exertion. On
examination she is pale, with a mild tachycardia of 105 beats per minute. She has mild pitting ankle
oedema. The results of her full blood count are given below.
Indices
Mrs Campbell's Results
Normal Range
Haemoglobin
78g/L
115-160g/L
MCV
106 fL
80 - 96 fL
MCH
33 pg
27-32pg
White cell count
3.8 x 109/L
4.0- 11.0 xl07L
Platelets
153 x 109/L
150 - 450 x 107L
42. Which one of the following nutritional deficiencies is most likely?
A) Iron
B) Magnesium
C) Thiamine
D) Folate
Answer D
43. With respect to Vitamin Bu and folic acid metabolism, which one of the following statements is
correct?
A) Vitamin Bi2 stores last longer than folic acid stores
B) Vitamin Bi2 is absorbed in the upper small bowel

C) Folic acid requires a gastric co-factor for absorption
D) Vitamin B12 contains an atom of copper
Answer A
RFA2 SBA 2003
Case 7
Mrs Sarah van Hilst is a 39 year old clerk (three days per week) and also provides most of the care
of her four children (her husband works full-time and also spends three hours per day travelling to
and from work). Over the past 3 months, she has become increasingly tired and has also noticed
some shortness of breath and palpitations when walking up hills. She has experienced some
difficulty in swallowing - "food seems to stick in the throat" - in the past 6 to 8 weeks. Mrs van
Hilst's periods have always been "heavy", lasting 5 to 6 days every 28 days.
On examination, she looks quite pale, and her pulse is 100/minute, sinus rhythm. Her blood
pressure is 175/75 mm Hg. There is no abdominal tenderness or splenomegaly. Her fingernails are
spoon-shaped. You order a blood count, and the results are given below.
Indices
Hb
MCV
MCH
MCHC
WCC
Platelet count
Mrs van Hilst's Results

85g/L
69 fl
22 pg
31.9 g/dL
7.3 x 10y/L
380 x 10y/L
Normal Range
115-160g/L
80 - 96 fL
27 - 32 pg
32 - 36 g/dL
4.0-11.0 xlOy/L
150-450xl09/L
33. Given Mrs van Hilst's history, examination and findings, the most likely diagnosis is:
A) Aplastic anaemia
B) Pernicious anaemia
C) Iron deficiency anaemia
D) Auto-immune haemolytic anaemia
Answer C
Curriculum Reference: 4.01 Lecture 4: Iron deficiency
Curator: A/Prof David Gottlieb (Haematology)
34. You go back to the blood film and examine it closely. The mostlikely finding is:
A) Hypochromic microcytic erythrocytes
B) Hypochromic macrocytic erythrocytes
C) Hyperchromic microcytic erythrocytes
D) Hypochromic spherocytic erythrocytes
Answer A

35. You decide to confirm your diagnosis with additional blood tests. If iron deficiency is present,
the set of results you would expect to find is:
A) Low serum iron, high serum transferrin, and low serum ferritin
B) Low serum iron, normal serum transferrin, and low serum ferritin
C) Low serum iron, high serum transferrin, and normal serum ferritin
D) Low serum iron, low serum transferrin, and high serum ferritin
Answer A
36. Iron absorption from food is facilitated by:
A) Iron being present as an organic compound
B) Iron being present in the form of ferrous (Fe2+) iron
C) Iron being present in the form of ferric (Fe3+) iron
D) Eating food derived from cereals such as wheat
Answer B
37. The largest amount of iron in the body is contained in:
A) Ferritin
B) Transferrin
C) Haemosiderin
D) Haemoglobin
Answer D
38. Erythrocytes in a person with iron deficiency are microcytic - i.e., smaller than normal - because
of:
A) Loss of intracellular water
B) Reduced intracellular haemoglobin concentration
C) An additional mitotic division during erythropoiesis
D) Impaired oxygen carrying ability

Answer C
Curriculum Reference: 4.04 Learning topicl: Microcytosis
39. When red cells reach the end of their life and are eliminated, the iron that they contain is:
A) Excreted in the urine
B) Excreted in the bile
C) Taken up by macrophages
D) Taken up by liver cells
Answer C
Case 8
Lily Klein is a 72 year old woman who presents for medical assessment prior to admission for hip
replacement. Her clinical examination reveals a 2 cm anterior cervical lymph node but no other
lymphadenopathy or hepatosplenomegaly. She is noted on full blood count to have a white cell
count of 25 x 109/L (reference range 4 - 11 x 109/L) with predominant lymphocytosis of 19.8 x 109/L
(reference range 1.5 - 4.0 x 109/L), but normal haemoglobin and platelets. The blood film comment
states: consistent with chronic lymphocytic leukaemia.
41. Regarding T-lymphocytes, which one of the following is NOT correct? They:
A) Secrete antibodies into the circulating blood
B) Interact with macrophages by secreting interleukins
C) Attack virus-infected cells by releasing perforin
D) Attack transplanted cells displaying non-self antigens
Answer A
Curriculum reference: 4.02 LT2
Author: L. Cottee, Physiology
43. With respect to chronic myeloid leukaemia (CML) and / or chronic lymphocytic leukaemia
(CLL)
which one of the following is correct?
A) CML typically exhibits a characteristic chromosomal translocation
B) CLL patients have no increase in bacterial infections
C) CLL can always be distinguished from lymphomas by examination of blood films
D) In patients with CLL the incidence of carcinomas of skin and lung is not increased
Answer A
Curriculum reference: 4.02 Lecture 2 Curator:
A/Prof David Gottlieb (Haematology)
Case 9
Ms Malini Mokashi is a 30 year old woman who is admitted to hospital with multiple trauma
following a motor vehicle accident. She has a previous history of blood transfusion following a
postpartum haemorrhage 5 years ago. There has been considerable blood loss and she is found to be
hypotensive and tachycardic in the Emergency Department. A sample for grouping and
crossmatching is sent to the laboratory with an urgent request for 6 units of blood.
45. Blood grouping is performed and Ms Mokashi is found to be group O Rh(D) negative. An
antibody screen is negative. Which one of the following statements regarding provision of blood for
the patient is correct?
A) A further full crossmatch is required against the selected units to be transfused before
blood can be issued
B) Whole blood should be used in preference to packed cells
C) Group-specific blood can be issued without further testing
D) If group O Rh(D) negative blood is not immediately available, O Rh(D) positive blood
should not be given
Answer C
Curriculum Reference: 4.01BCS Theme Session 2: Laboratory skills: group, X-match.
4.03 Lecture 4: (Blood products in the treatment of disease)
Over the course of the next 24 hours, Ms Mokashi is given 20 units of blood before surgical
stabilisation of injuries is achieved. It is noted that there is continuing oozing from wound sites and
cannula punctures.
46. The most likely cause for this is:
A) Dilution of clotting factors and platelets
B) Liver failure as a result of injury and hypotension
C) Disseminated intravascular coagulation
D) Excessive amounts of citrate anticoagulant in transfused blood
Answer A
Curriculum Reference: 4.03 Lecture 4 (Blood products in the treatment of disease)
47. Which one of the following products is most appropriate for replacing coagulation factors?
A) Platelet concentrates
B) Whole blood
C) Fresh frozen plasma
D) Cryoprecipitate
Answer C
Curriculum Reference: 4.03 Learning Topic 8: Acquired disorders of clotting factors.
52. In P-thalassaemia major there is:
Answer C
Curriculum references: 4.02 Lect 1,4 Curator:
53. Antenatal diagnosis of P-thalassaemia major is most commonly made by performing:
A) A full blood count on fetal blood
B) Haemoglobin electrophoresis on fetal blood
C) DNA molecular techniques on a chorionic villus sample
D) a/p globin synthesis ratios on chorionic villus sample
Answer C
Curriculum reference: 4.04 Lecture 4 Curator:
Case 10
Alexa Dimitriou, a 9 month old girl, is brought into your general practice by her mother and
grandmother. They have just migrated to Australia from Greece. She presents with a one month
percentile for age, she is pale, has marked hepatosplenomegaly and a hyperdynamic circulation.
Full blood count findings include:
Hb 45 g/L
(normal range for this age 95 - 140 g/L)
MCV 68fl
(normal range for this age 70 - 85 fl)
You consider a diagnosis of thalassaemia.
50. In chronic anaemia:
A) The cardiac output is reduced

B) The symptoms will be greater than in acute anaemia
C) Red cell 2,3-diphosphoglycerate is increased
D) The heart rate is decreased
Answer C
Curriculum references: 4.05 LT7, 4.01 LT5, 4.02 BCS1
51. If Alexa has thalassaemia major as you suspect, her blood film will show:
Answer A
Case 13
Marianne Issa is a 4 year old girl who is noted on routine medical examination to have a heart
murmur. She is referred to a paediatric cardiologist who diagnoses a small ventricular septal
defect.
66. Should Marianne be suffering bacterial endocarditis, the most likely causative agent is:
A) A species of oral streptococci
B) Staphylococcus aureus
C) Coagulase negative staphylococci
D) A Gram-negative (enteric) rod
Answer A
Curriculum reference: 4.03Lec 1 .-Rheumatic valve disease; LT.6 Complications of rheumatic heart
disease
Author: A/Prof R. Kearney, Infectious diseases
RFA2 SBA 2004
Case 10
Max Friedrichs is an alert three year old boy who has been recently diagnosed with haemophilia A.
46. A diagnosis of haemophilia A is suggested by:
1 A) A reduced factor IX level
2 B) A factor XIII level of <10%
3 C) Reduced levels of von Willebrand factor
4 D) A long activated partial thromboplastin time (APTT)
Answer D
PBL Reference: 4.03, LT1,:LT2, LT3, Lec 1
47. The replacement product of choice for a newly-diagnosed boy with Haemophilia A is:
1 A) Recombinant factor VIII
2 B) Cryoprecipitate
3 C) AHF (anti-haemophilia factor)
4 D) Fresh frozen plasma
Answer A
PBL Reference: 4.03, Lec3, Lec 4
48. The prothrombin time (PT) is prolonged in association with:
1 A) Blood transfusion
2 B) Heparin therapy
3 C) Severe thrombocytopenia
4 D) Warfarin therapy
Answer D
PBL Reference: 4.03, LT8, Lec 1
49. In the coagulation cascade, which of the following factors, when activated, cleaves prothrombin
to thrombin?
1 A) Factor XI
2 B) Factor X
3 C) Factor IX
4 D) Factor VIII
Answer B
Activated factor X, which is generated from either pathway, activates prothrombin to thrombin,
which in turn leads to conversion of fibrinogen to fibrin which serves to stabilize the platelet plug.
Curriculum References: 4.03 LT1
50. A female carrier for classical haemophilia marries a close relative with classical haemophilia.
The risk of a female infant being affected with haemophilia is:
1 A) 1.0
2 B) 0.5
3 C) 0.25
4 D) zero
Answer B
Female offspring will either be X*X* (haemophiliac) or X*X (carrier). The phenotype will depend
upon the nature of the factor VIII mutation but defective function of factor VIII is likely given that
X*Y has induced haemophilia in the father.
Curriculum reference: 4.03 LT6 Genetics of haemophilia
51. Non-steroidal drugs, such as aspirin, reduce platelet aggregation because they:
1 A) Inhibit megakaryocyte formation in bone marrow
2 B) Cause immune thrombocytopenic purpura
3 C) Block GPIIIa receptors on platelets
4 D) Inhibit the formation of thromboxane A2 in the platelets
Answer D
Curriculum reference: 4.03 LT7 Platelet dysfunction
Case 11
Lazika Merken, a ten month old girl, is brought into your general practice by her parents. The family
have just migrated to Australia from east coast of Turkey. Lazika has a six week history of poor
feeding. On examination her weight is on the 3rd percentile for age, she is pale, has marked
hepatosplenomegaly and a hyperdynamic circulation.
Hb 47 g/L (normal range for this age 95 - 140 g/L)
MCV 68fl (normal range for this age 70 - 85 fl)
52. If Lazika has thalassaemia major, her blood film will show:
1 A) Macrocytic red cells
2 B) Spherocytic red cells
3 C) Hypochromic red cells
4 D) Hyperchromic red cells
Answer C
53. In -thalassaemia major there is:
1 A) Normal production of abnormal beta chains
2 B) Inadequate production of normal beta chains
3 C) Inadequate production of normal alpha chains
4 D) Normal production of abnormal alpha chains
Answer B
54. Antenatal diagnosis of -thalassaemia major is most commonly made by performing:
1 A) A full blood count on fetal blood
2 B) Haemoglobin electrophoresis on fetal blood
3 C) / globin synthesis ratios on a chorionic villus sample
4 D) DNA molecular techniques on a chorionic villus sample
Answer D
Curriculum reference: 4.04 Lect 4
Case 12
Howard Johnson is a 75 year old man who attends your general practice usually only for his annual
influenza vaccination. Today he complains of a cough and runny nose over the last few days. He is
afebrile and auscultation of his chest is normal. On further examination you notice pronounced
enlargement of lymph nodes over all of his body. The full blood count reveals a total white cell count
of 92.0 x 109/L, containing predominantly lymphocytes.
55. What is the most likely underlying diagnosis in this man?
1 A) Influenza
2 B) Non-Hodgkins lymphoma
3 C) Infectious mononucleosis
4 D) Chronic lymphocytic leukemia
Answer D
PBL reference: 4.04 Lecture 2 (Introduction to lympho-proliferative disease)
56. Which one of the following is NOT generally associated with lymphadenopathy?
1 A) Myelofibrosis
2 B) Sarcoidosis
3 C) Tuberculosis
4 D) Epstein Barr virus
Answer A
PBL reference: 4.04 Learning Topic 1 (Lymphadenopathy)
57. In normal lymph nodes:
1 A) Immunoglobulin genes can mutate following antigenic stimulation
2 B) Germinal centres contain predominantly plasma cells
3 C) Follicles contain predominantly T cells
4 D) Parafollicular zones contain predominantly B cells.
Answer A
PBL reference: 4.04 Lecture 2 (Introduction to lympho-proliferative disease)
One year after diagnosis, Mr Johnson returns with symptoms of lethargy, palpitations and dyspnoea
on exertion. He has noted dark urine for one week. Blood tests show the following:
Haemoglobin 70 g/L (normal range: 130 182 g/L)
MCV 92 fL (normal range: 80 96 fL)
MCH 31.2 pg (normal range: 27 32 pg)
White cell count 120 x 109/L (normal range:4.0 11.0 x 109/L)
Differential: Neutrophils 10 x 109/L, lymphocytes 108 x 109/L and monocytes 2 x 109/L
Platelets 545 x 109/L (normal range: 150 450 x 109/L)
ESR 68 mm/hour
Direct Antiglobulin Test - Strongly positive
58. What is the best interpretation of these results?

1 A) Anaemia of chronic disease
2 B) Iron deficiency anaemia
3 C) Hereditary spherocytosis
4 D) Autoimmune haemolytic anaemia
Answer D
PBL reference: 4.01 Learning Topic 4 (Premature destruction of red cells); 4.04 Learning Topic 3
(Autoimmunity causing haematological disorders)
1 A) Red cell 2,3-diphosphoglycerate is increased
2 B) The heart rate is decreased
3 C) The cardiac output is reduced
4 D) The symptoms will be greater than in acute anaemia
Answer A
60. The therapeutic option of most likely benefit would be:
1 A) Plasmapheresis
2 B) Corticosteroid therapy
3 C) Intravenous iron
4 D) Folic acid supplementation
Answer B
PBL reference: 4.01 Learning Topic 4 (Premature destruction of red cells); 4.04 Lecture 2
(Introduction to lympho-proliferative disease)
Mr Johnsons condition deteriorated, with increasing left upper quadrant pain, and a falling
haemoglobin level. A CT scan was performed which showed splenic rupture with intra-abdominal
haemorrhage. A splenectomy was perfomed.
61. Which one of the following statements is correct with respect to splenectomy?
1 A) Post-splenectomy patients are susceptible to infection by unencapsulated bacteria
2 B) An increase in blood leucocytes can occur after a splenectomy
3 C) Post-splenectomy patients have increased resistance to malaria
4 D) Portal hypertension can occur as a complication of splenectomy
Answer B
PBL reference: 4.02 Lecture 3 (Diagnosis of malaria); 4.04 Learning Topic 4 (Immunodeficiency
and common opportunistic infections)
Case 14
Mary Takatis is a 83 year old woman who presents to the Emergency Department with acute onset of
breathlessness due to pulmonary oedema. 12 lead ECG is normal apart from a sinus tachycardia. A
diagnosis of heart failure is made.
68. Which one of the following factors will increase the likelihood of tissue oedema occurring?
1
A) An increase in total peripheral resistance
2
B) An increase in peripheral venous pressure
3
C) An increase in plasma protein concentration
4
D) An increase in cardiac output
Answer B
An increase in peripheral venous pressure will result in an increase in capillary hydrostatic
pressure, favouring filtration. An increase in total peripheral resistance tends to decrease pressure in
the capillaries, favouring reabsorption. An increase in plasma protein concentration also favours
reabsorption.
Ref: 4.03 Oedema (Learning topic)
RFA2 SBA 2005
Case 10
John OSullivan, a 65 year old man, presented to his GP with several lumps under both axillae. He
said that he has felt more lethargic in the last 18 months. Although previously very well, he has
experienced several episodes of severe upper respiratory tract infections in the last 6 months. On
examination, he had generalized lymphadenopathy in the cervical, axillary and inguinal regions, and
splenomegaly of 5 cm below the costal margin.
The blood film showed a lymphocytosis with smudge cells. Results of a Full Blood Count are given
in the table below.
Indices
Haemoglobin
White cells
Neutrophils
Lymphocytes
Platelets
Mr OSullivans results
100 g/L
73 x 109/L
2.5 x 109/L
70 x 109/L
73 x 109/L
Normal range
130 180 g/L
4.0 11 x 109/L
2.0 7.5 x 109/L
1.5 4.0 x 109/L
150 400 x 109/L
50. With respect to splenomegaly, which one of the following is correct?

A)
B)
C)
D)
Increased red cell destruction contributes to the associated anaemia

Massive enlargement is indicative of malignant disease
Pancytopenia is invariably present
Red cell inclusions (Howell-Jolly bodies) are frequently seen in peripheral blood
films
Answer A
Explanation:Accelerated red cell destruction is the major mechanism responsible for the anaemia of
splenomegaly. Pooling of red cells in the red pulp of the spleen and expansion of plasma volume
producing a dilutional component, are additional mechanisms. Massive splenic enlargement occurs
in a number of haematological malignancies, but also in some parasitic diseases (including
malaria), in congestive splenomegaly resulting from portal hypertension, and in some storage
diseases (eg Gauchers disease). In splenomegaly there may be a reduction in the number of
circulating red cells, leucocytes or platelets, alone or in any combination. Pancytopenia is not
invariable. Howell-Jolly bodies are seen after splenectomy and in hyposplenism, not in
splenomegaly.
PBL Reference: 4.04 L T 2 Splenomegaly
51. The most likely cause of the enlarged spleen in Mr OSullivan is:
A)
B)
C)
D)
Infiltration by neutrophils
Recurrent upper respiratory tract infections
Infiltration by platelets
Infiltration by lymphocytes
Answer D
Curriculum reference: 4.04 L T 2 Splenomegaly
52. The most likely cause of Mr OSullivans anaemia is:
A)
B)
C)
D)
Bone marrow infiltration

Haemolysis
Iron deficiency
Myelodysplasia
Answer A
Explanation; Marrow infiltration by lymphocytes replaces the normal cellular elements, including
erythroblasts. Autoimmune haemolysis can occur in CLL, but is less common. CLl does not directly
cause iron deficiency and does not arise from myelodysplasia
PBL Reference:4.02, Lec 2 Leukemia; LT1 Lymphadenopathy
53. Which one of the following is most likely to contribute to his recurrent infections?
A)
B)
C)
D)
Tonsillar atrophy
Hypogammaglobulinaemia
Hyperviscosity caused by the lymphocytosis
Neutropenia
Answer B
The abnormal lymphocyte population in CLL leads to a generalised deficiency of gamma globulins,
which is an important risk factor for recurrent bacterial infections, particularly of the respiratory
tract. Lymphocytes, being small, do not cause hyperviscosity until the counts are extremely high
(>300). The patients current neutrophil count is within normal limits.
PBL Reference: 4.02, LT7 Immunodeficiency and common opportunistic infections
Case 11
Oliver Lee is a 5 year old boy who has been brought in by his parents who thought he was often
listless and tired. The family is of South-East Asian origin. You are the family doctor. You think
Oliver looks pale and you perform a series of blood tests.
The blood film showed hypochromic microcytosis, with target cells, tear drop poikilocytes and
basophilic stippling. You think thalassaemia is possible.
The following results were found:
HbA2
HbF
HbEPG
HbH bodies
3.5 % (normal range 1.5 - 3.5%)

96.5 % (normal range < 1%)
no abnormal bands
not detected
54. Which of the following is correct concerning haemoglobin?

A)
B)
C)
D)
It features an oxygen binding, copper-chelated porphyrin-ring functional group

It is composed of several globin molecules each containing a single haem moiety
It may be formed by co-existing beta and delta chains
Has increased affinity for oxygen in the presence of 2,3-diphosphoglycerate (DPG)
Answer B
Haem is an iron-chelated porphyrin ring structure; 2,3-DPG decreases oxygen binding thereby
releasing oxygen into the tissues.
56. HbF:
A)
B)
C)
D)
Is made up of 22 tetramers
Has a higher oxygen affinity than adult haemoglobin
Forms the major fraction of total haemoglobin during late infancy
Is the only haemoglobin present in the first two trimesters of gestation
Answer B
HbF (22) has a higher oxygen affinity than maternal haemoglobin which facilitates the transfer of
oxygen from the mother to the fetus. At birth HbF represents 70 90% of total haemoglobin, by 6
months it represents only 5%, it gradually reduces to the adult level of <1% during childhood.
Other haemoglobins are present during the first two trimesters including Gower1, Gower 2 and very
small amounts of adult haemoglobin (HbA).
Reference: 4.04 LT6 Structure of normal haemoglobins; LT7 Abnormalities of haemoglobin genes
57. The basic defect seen in patients with -thalassaemia major is:
A)
Excess production of a structurally normal -globin chain
B)
C)
D)
Excess production of a structurally abnormal -globin chain

Normal production of a structurally abnormal -globin chain
Inadequate production of a structurally normal -globin chain
Answer D
-thalassaemia major is due to a reduced rate of production of structurally normal -globin
Reference: 4.04, LT 5 Shortened red cell survival; LT6 Structure of normal haemoglobins; LT7
Abnormalities of haemoglobin genes
58. Antenatal diagnosis of -thalassaemia major is most commonly made by performing:
A)
B)
C)
D)
A full blood count on fetal blood

Haemoglobin electrophoresis on fetal blood
DNA molecular techniques on a chorionic villus sample
/ globin synthesis ratios on chorionic villus sample
Answer C
Curriculum reference: 4.04 Lecture 4 Prenatal diagnosis of thalassaemia
Case 12
During her first pregnancy 3 months ago, Melissa Simms, a 25 year old woman, suffered an
intrauterine death at a gestation of 28 weeks. Her periods have not yet restarted. She is seen by her
general practitioner 3 weeks after discharge from hospital, complaining of fatigue and exertional
breathlessness. Blood tests were performed, and were consistent with a diagnosis of iron deficiency
anaemia.
59. Which one of the following is characteristic of pregnancy?
A)
B)
C)
D)
The placenta produces inhibitors of maternal red cell production

There is reduction in the circulating fibrinogen level
There is decreased folate absorption
There is a substantial increase in iron requirements
Answer D
A. False: The placenta does not produce inhibitors of maternal red cell production.
B. False: The fibrinogen level, as well as those of several other coagulation factors, rises in
pregnancy, leading to a thrombotic tendency.
C. False: Folate absorption increases in pregnancy.
D. True: The major causes of anaemia in pregnancy are dilutional. An additional 800-1000 mg iron
is required to meet the demands of pregnancy, 1/3 for the fetal-placental unit, and 2/3 for the
increased red cell mass.
Curriculum Reference: 4.05 LT5 Hormonal effects on blood
Ms Simms' menstrual cycles began at age 13 years, with regular cycles of 28 days, but up to 7 days
bleeding, with heavy flows for the first 3 days, and with some dysmenorrhoea. Oral contraceptives
have been used in the past to control these problems. She is otherwise well, has no other significant
medical or surgical history, and has no gastrointestinal symptoms. There is no family history of
anaemia or other blood disorders.
60. Which one of the following statements summarizes the most likely cause of this patients
anaemia?
A)
B)
C)
Iron deficiency due to heavy periods

Poor dietary intake of iron, especially since the induced labour
Recent post-partum bleeding, together with pre-existing iron deficiency and increased
demand for iron during pregnancy
D) Failure to take folate supplements during the pregnancy.
Answer C
A. False: The cause of the anaemia is most likely to be multi-factorial. Iron deficiency due to heavy
periods is possible, as she required the OC pill to control menorrhagia. However other factors are
also contributory.
B. False: No information is given to suggest that her diet has been poor in iron since the
miscarriage, and in any case the cause of the anaemia is most likely to be multi-factorial.
C. True: In addition to possible pre-existing iron deficiency, she suffered a post-partum
haemorrhage, and the pregnancy itself would have increased her requirements for iron.
D. False: Folate deficiency would result in a macrocytic anaemia, whereas this woman has the
characteristic microcytic hypochromic anaemia associated with iron deficiency.
Curriculum reference: 4.01 Lect 4 Iron deficiency; 4.05 LT5 Hormonal effects on blood
61. Which one of the following series of results is consistent with iron deficiency?
A)
B)
C)
D)
Elevated serum ferritin, reduced serum iron, and normal serum transferrin
Normal serum ferritin, reduced serum iron, and reduced total iron binding capacity
Reduced serum ferritin, elevated serum transferrin, and absent stainable marrow iron
Elevated serum ferritin, normal iron saturation, and stainable marrow iron
Answer C
A. False: This pattern suggests anaemia of chronic disease.
B. False: This pattern suggests anaemia of chronic disease.
C. True: This combination is typical of iron deficiency.
D. False: Ferritin is an acute phase reactant and therefore the serum ferritin may be spuriously
elevated in other conditions such as infection, inflammation and neoplasia.
Curriculum reference: 4.05 LT7 Tests of iron status in anaemia.
62. Which one of the following statements is correct?
A)
B)
C)
D)
Re-utilization of iron from phagocytosed red cells prevents iron deficiency

The majority of absorbed iron is utilized by myoglobin
Iron balance is primarily controlled by the rate of iron excretion in men
Vitamin C reduces iron absorption when iron is in the ferrous form
Answer A
A. True: The daily requirements of iron exceed the amount that is absorbed
B. False: The majority of absorbed iron is utilized by haemoglobin.
C. False: Iron absorption is the primary mechanism controlling iron balance.
D. False: Vitamin C enhances iron absorption by converting iron from ferric to ferrous.
Curriculum reference: 4.05 LT6 Iron metabolism
63. Which one of the following proteins is the major carrier of iron in blood?
A)
B)
C)
D)
Albumin
Ferritin
Transcobalamin
Transferrin
Answer D
Curriculum reference: 4.01 LT3 Consequences of deficiency
RFA3SBA2001
Case 6
Alexa Dimitriou, a 9 month old girl, is brought into your general practice by her mother and
grandmother. They have just migrated to Australia from Greece. She presents with a one month
percentile for age, she is pale, has marked hepatosplenomegaly and a hyperdynamic circulation.
Hb 45 g/L
(normal range for this age 95 - 140 g/L)
MCV 68fl
(normal range for this age 70 - 85 fl)
You consider a diagnosis of thalassaemia.
32. With respect to the liver, which one of the following statements is correct?
A) The left and right anatomical lobes are demarcated on the basis of their blood supply
B) The hepatic vein drains to the porta hepatis
C) The ligamentum teres connects the liver to the umbilicus
D) The normal adult liver lies entirely under cover of the lower thoracic cage
Answer C
The right and left functional lobes are demarcated on the basis of their blood supply. The anatomical
lobes are demarcated by the falciform ligament anteriorly and the fissures for ligamentum venosum
and teres posteriorly. The portal vein drains to the porta hepatis, while the hepatic vein drains to the
inferior vena cava. The liver is not covered by the thoracic cage in the midline, where it lies deep to
muscles of the anterior abdominal wall.
Author: D Bryce, Anatomy
Curriculum reference: 7.11 Learning topic, 7.08 Theme session notes
A) The cardiac output is reduced
B) The symptoms will be greater than in acute anaemia
C) Red cell 2,3-diphosphoglycerate is increased
D) The heart rate is decreased
Answer C
In chronic anaemia the compensatory mechanisms include an increased cardiac output, increase
heart rate, a shift in the oxygen dissociation curve so that more oxygen is released to the tissues.
This shift in the oxygen dissociation curve is due to an increase in the amount of 2.3DPG in the red
cells. Because of these compensatory mechanisms, chronic anaemia even of a severe degree is
surprisingly well tolerated.
34. If Alexa has thalassaemia major as you suspect, her blood film will show:
Answer A
The blood film in thalassaemia major shows hypochromic usually microcytic cells with marked
anisocytosis (variation in size) and poikilocytosis (variation in shape) associated with the presence
of nucleated red blood cells. Spherocytes are not a feature of thalassaemia major and are typically
seen in hereditary spherocytosis and immune haemolytic anaemia.
35. In b-thalassaemia major there is:
Answer C
In beta thalassaemia major there is inadequate production of normal beta globin chains

36. Antenatal diagnosis of b-thalassaemia major is most commonly made by performing:
A) A full blood count on fetal blood
B) Haemoglobin electrophoresis on fetal blood
C) DNA molecular techniques on a chorionic villus sample
D) a/b globin synthesis ratios on chorionic villus sample
Answer C
Antenatal diagnosis of beta thalassaemia major is most commonly performed by molecular
techniques (eg RFLP analysis) on a CVS sample.
Curriculum reference: 4.04 Lect 4
Case 7
Lily Klein is a 72 year old woman who presents for medical assessment prior to admission for hip
replacement. Her clinical examination reveals a 2 cm anterior cervical lymph node but no other
lymphadenopathy or hepatosplenomegaly. She is noted on full blood count to have a white cell count
of 25 x 109/L (reference range 4 - 11 x 109/L) with predominant lymphocytosis of 19.8 x 109/L
(reference range 1.5 - 4.0 x 109/L), but normal haemoglobin and platelets. The blood film comment
states: consistent with chronic lymphocytic leukaemia.
37. Regarding T-lymphocytes, which one of the following is NOT correct? They:
A) Secrete antibodies into the circulating blood
B) Interact with macrophages by secreting interleukins
C) Attack virus-infected cells by releasing perforin
D) Attack transplanted cells displaying non-self antigens
Answer A
Author: L. Cottee, Physiology Curriculum
reference: 4.02 LT2
38. The thoracic duct:
A) Is located in the posterior mediastinum
B) Normally drains into the azygos vein
C) Passes through the diaphragm with the inferior vena cava
D) Drains directly into the spleen
Answer A
The thoracic duct conveys most lymph of the body to the venous system. It ascends from the cisterna
chyli through the aortic hiatus in the diaphragm, ascends in the posterior mediastinum on the bodies
of the thoracic vertebrae. It empties into the venous system near the union of the left internal jugular
and subclavian veins.
Author: Bill Webster, Anatomy Curriculum

reference: 7.08 LT7
39. With respect to chronic myeloid leukaemia (CML) and / or chronic lymphocytic leukaemia
(CLL) which one of the following is correct?
A) CML typically exhibits a characteristic chromosomal translocation
B) CLL patients have no increase in bacterial infections
C) CLL can always be distinguished from lymphomas by examination of blood films
D) In patients with CLL the incidence of carcinomas of skin and lung is not increased
Answer A
Chronic myeloid leukaemia in 95% of patients carries the Philadelphia chromosome produced by
translocation 9;22. Although chromosomal abnormalities are found in over half of patients with
chronic lymphocytic leukaemia there is no one uniform or constant chromosome abnormality.
Chronic lymphocytic patients have an increased incidence of all infections due both to opportunistic
and bacterial pathogens. Moreover chronic lymphocytic leukaemia may be indistinguishable from
small lymphocytic lymphoma (in leukaemic phase) both on clinical examination and on blood film.
Second malignancies (carcinomas at various sites) are increased in patients with chronic
lymphocytic leukaemias.
Author: J Wiley, Haematology Curriculum
reference: 4.04 Lec 3
40. Which one of the following infections is commonly associated with severe neutropaenia from
leukaemia chemotherapy?
A) Listeria monocytogenes
B) Mycobacterium tuberculosis
C) Pseudomonas aeruginosa
D) Herpes simplex virus
Answer C
The organism P. aeruginosa is a commonly isolated Gram-negative rod from CLL patients with
neutropaenia. The organism gains access to the circulation when rapidly multiplying intestinal
epithelium is damaged by chemotherapy. The organisms A, B and D are more likely where there is a
T cell immune deficit, rather than neutropaenia.
Author: A/Prof. R. Kearney, Infectious diseases
Curriculum reference:
LT 4.04 Immunodeficiency and common opportunistic infections
L 1.08 Intracellular infections
TS. 1.08 Intracellular infections/chronic inflammation and TB
RFA3SBA2002
Case 6
Nicholas O'Brien is an 18 month old boy, brought to the Emergency Department by his mother. He
is bleeding from the mouth. History taking reveals that he fell over two days previously and cut his
mouth. His mother gave him some ice blocks and the bleeding eventually stopped several hours
later. However, it recurred that evening, and he has been bleeding on and off ever since. You
consider the possibility that he has a bleeding disorder.
26. Which one of the following statements is correct? If Nicholas has
A) Haemophilia A, then his father is likely to be a carrier of the disease
B) Haemophilia B, then his mother is likely to be a carrier of the disease
C) Von Willebrand's disease, then his sister has a 1 in 4 chance of also suffering from von
Willebrand's disease
D) Haemophilia A, the causative mutation lies on chromosome 21
Answer B
A. False: Haemophilia A is an X-linked recessive disorder. If Nicholas has inherited haemophilia A,
he did so from his mother.
B. True: Haemophilia B is also an X-linked recessive disorder, so his mother is likely to be a
carrier.
C. False: Von Willebrand's disease is inherited as an autosomal dominant disorder, and therefore
his
sister has a 1 in 2 chance of also inheriting the disease.
D. False: Haemophilia A is due to mutations in the factor VIII gene which is found on theX
chromosome.
Curriculum Reference: 4.03, LT3, LT6, BCS2
Author: John Rasko & Harry Hand, Haematology
The laboratory investigations shown yield the following results:
Indices
Hb
WCC
Platelet count
APTT (activated partial thromboplastin
PT (prothrombin time)
Renal and hepatic function
Results
115 g/L
10.5xl09/L
260 x 109/L
*75 seconds
13 seconds
normal
Reference Range
105-140 g/L
6.0-18.0 x 109/L
150-500 x 109/L
25-35 seconds
11-15 seconds
27. Which one of the following is most correct?

A) Assays for coagulation factors VIII and IX should be performed
B) Von Willebrand's disease is unlikely because the platelet count is normal
C) Haemophilia A is unlikely because the prothrombin time is normal
D) If Nicholas has haemophilia B, his platelet function is likely to be abnormal
Answer A
A. True: The presence of a significantly prolonged APTT with normal PT suggests a defect in the
intrinsic pathway. Together with the history, the prolonged APTT indicates haemophilia A,
haemophilia B or von Willebrand's disease are likely diagnoses. Therefore factors VIII and IX and
von Willebr and factor should be assayed.
B. False: Most patients with von Willebrand's disease have a normal platelet count even though
platelet function is abnormal.
C. False: The prothrombin time tests the extrinsic pathway, whereas haemophilia A is due to
reduced
levels of factor VIII which is in the intrinsic pathway.
D. False: Platelet function tests are normal in both haemophilia A and haemophilia B.
Curriculum Reference: 4.03, Lecture 1, LT5, BCS1, BCS2 Author:
John Gibson, Kevin Richard & Harry Hand, Haematology
28. In the coagulation cascade, which of the following factors, when activated, cleaves prothrombin
to thrombin?
A) Factor VIII
B) Factor IX
C) Factor X
D) Factor XI
Answer C
Activated factor X, which is generated from either pathway, activates prothrombin to thrombin,
which in turn leads to conversion of fibrinogen to fibrin which serves to stabilize the platelet plug.
Curriculum References: 4.03 LT1 Author:
Julie Curtin, Haematology
29. Vitamin K acts as a coenzyme for a reaction in which the clotting factors II, VII, IX and X are
modified by carboxylation at glutamate residues. This modification has the effect of sensitizing
these
clotting factors to:
A) Calcium ions
B) Protein C
C) Thrombin
D) Warfarin
Answer A
gamma-carboxyglutamate residues in factors II, VII, IX and X act as binding sites for calcium ions
(factor IV).
Curriculum Reference: 4.03 LT2 Physiology of clotting factors
Author: A Conigrave, Biochemistry
30. Some children with haemophilia also experience problems in psychosocial development. Which
one of the following is indicative of psychosocial dysfunction in children aged 5 to 12 years?
A) A growth in dependence on peers
B) Acceptance of rules at school and in games
C) A friendship network of less than five
D) Little or no reduction in egocentricity from preschool levels
Answer D
There is normally a reduction in egocentricity during the middle childhood years as children
develop emotionally, becoming more aware of the differences between themselves and other people.
This emotional development is marked by substantial gains in perspective-taking and empathy.
Curriculum Reference: 4.03 Lect 4 "Psychosocial aspects of haemophilia"
Author: Dr Di Sansom, Behavioural Sciences
Case 11
Nathan Leung is a 5 year old boy who has been brought in by his parents who thought he was often
listless and tired. The family is of South-East Asian origin. You are the family doctor. You think
Nathan looks pale and you perform a series of blood tests.
The blood film showed hypochromic microcytosis, with target cells, tear drop poikilocytes and
basophilic stippling. You think thalassaemia is possible. The following results were found:
HbA2
HbF
HbEPG
HbH bodies
3.5 % (normal range 1.5 - 3.5%)

96.5 % (normal range < 1%)
no abnormal bands
not detected
51. Haemoglobin:
A) Features an oxygen binding, copper-chelated porphyrin-ring functional group
B) Is composed of several globin molecules each containing a single haem moiety
C) May be formed by co-existing beta and delta chains
D) Affinity for oxygen increases in the presence of 2,3-diphosphoglycerate (DPG)
Answer B
Haem is an iron-chelatedporphyrin ring structure; 2,3-DPG decreases oxygen binding thereby
releasing oxygen into the tissues.
Curriculum Reference: 4.04 LT6 Structure of normal haemoglobins
52. The basic defect seen in patients with (3-thalassaemia major is:
A) Excess production of a structurally normal (3-globin chain
B) Excess production of a structurally abnormal (3-globin chain
C) Normal production of a structurally abnormal |3-globin chain
D) Inadequate production of a structurally normal (3-globin chain
Answer D
f3-thalassaemia major is due to a reduced rate of production of structurally normal /3-globin
Curriculum Reference: 4.02, Learning Topic 5 (Shortened red cell survival)
4.02, Learning Topic 6 (Structure of normal haemoglobins) 4.02, Learning
Topic 7 (Abnormalities of haemoglobin genes) Author: Julie Curtin,
Paediatrics & Child Health
53. HbF:
A) Is made up of a2o2 tetramers
B) Has a higher oxygen affinity than adult haemoglobin
C) Forms the major fraction of total haemoglobin during late infancy
D) Is the only haemoglobin present in the first two trimesters of gestation
Answer B
HbF (a2y2) has a higher oxygen affinity than maternal haemoglobin which facilitates the transfer of
oxygen from the mother to the fetus. At birth HbF represents 70 - 90% of total haemoglobin, by 6
months it represents only 5%, it gradually reduces to the adult level of <1% during childhood.
Other haemoglobins are present during the first two trimesters including Gowerl, Gower 2 and very
small amounts of adult haemoglobin (HbA).
Curriculum Reference: 4.02, Learning Topic 6(Structure of normal haemoglobins)
4.02, Learning Topic 7 (Abnormalities of globin gens) Author: Julie Curtin,
Paediatrics & Child Health
RFA3SBA2004
Case 6
Mr Nayudu is a 53 year old man who presents to his GP with mild dyspnoea on exertion and a
history suggestive of angina. There is no history of blood loss. Results of a full blood count are
given in the table below. An occult blood test was performed on a stool specimen and reported to be
negative.
Indices
Haemoglobin
MCV
MCHC
White Cell Count
Platelets
Mr Nayudu's Results
75 g/L
65.3 fL
317 g/L
4.7 x 107L
426 x 10y/L
Normal Range
130 -180 g/L
80-100fL
300-350 g/L
4.0-11.0x10^
150-400xl07L
26. Which one of the following disorders may manifest as hypochromic microcytic anaemia?
A) Haemolytic anaemia
B) Thalassaemia trait
C) Liver disease
D) Acute blood loss
Answer B
PBL reference: 4.01 Lee 4 Iron deficiency; 4.04 LT1 Microcytosis
Author: James Isbister, Haematology
27. Which one of the following series of results would be consistent with iron deficiency?
A) Increased transferrin saturation with iron, reduced serum ferritin, and normal serum
transferrin
B) Reduced transferrin saturation with iron, reduced serum iron, and reduced total iron
binding capacity
C) Reduced serum ferritin, reduced iron saturation, and increased total iron binding
capacity
D) Reduced serum transferrin and reduced serum transferrin receptor
Answer C
A-False: This pattern suggests haemochromatosis.
B-False: This pattern suggests anaemia of chronic disease.
C-True: This combination is typical of iron deficiency.
D-False: Serum transferrin and transferrin receptor levels are typically elevated in iron deficiency.
PBL reference: 4.05, LT7 Tests of iron status in anaemia
Author: Prof Paul Vincent, (RPAH) -adapted by C. Ward
28. Which one of the following is correct about iron?
A) Iron is more readily absorbed in the ferric (Fe3+) form
B) Iron absorption is enhanced in the presence of ascorbate
C) Transferrin is the main reservoir of iron in the liver
D) Ferritin represents the major circulating form of iron
Answer B
The ferrous form of iron is more readily absorbed and iron is stored as ferritin, not transferrin.
Transferrin is the major circulating iron binding protein.
PBL reference: 4.05 LT6 Iron metabolism
Author:Dr Chris Ward, Dept of Haematology (RNSH)
29. The transfer of oxygen from haemoglobin to the tissues is:
A) Decreased by 2,3-diphosphoglycerate (2,3-DPG)
B) Increased at low pH
C) Not affected by the storage of red cells at 4C
D) Enhanced by fetal haemoglobin
Answer B
2,3-DPG lowers the affinity of haemoglobin for oxygen, thereby facilitating oxygen delivery to the
tissues. Acidosis (lower pH) lowers the affinity of haemoglobin for oxygen, resulting in a shift to the
right of the haemoglobin oxygen dissociation curve. The affinity for oxygen is greater in foetal than
in adult haemoglobin.
PBL Reference: 4.01, Lecture 1: Role of haemoglobin in oxygen delivery to tissues
Follow up blood tests confirm iron deficiency.
30. Which one of the following is the most likely cause of anaemia in Mr Nayudu?
A) Dietary iron deficiency
B) Malabsorption
C) Diverticulitis
D) Gastrointestinal malignancy
Answer D
PBL reference: 4.01 Lee 3 Introduction to anaemia
31. In the following donor recipient pairs, which of the red cell concentrates would be incompatible
and potentially cause a serious haemolytic transfusion reaction?
A) Donor Group O RhD Negative to Patient Group A RhD Positive
B) Donor Group A RhD Positive to Patient Group A RhD Negative
C) Donor Group AB RhD Positive to Patient Group B RhD Positive
D) Donor Group O RhD Positive to Patient Group AB RhD Positive
Answer C
PBL reference: BCS2 group x-match
RFA3SBA2005
Case 6
A Cambodian woman is admitted to hospital in premature labour at 32 weeks gestation with her first
pregnancy. Following development of fetal distress she undergoes an emergency Caesarian Section.
Her baby son is pale, and jaundiced, and has marked hepatosplenomegaly and ascites. FBC
demonstrates a haemoglobin of 60 g/L (NR 140 - 225 g/L). Blood film demonstrates hypochromic
red cells, target cells, polychromasia and nucleated red cells. Haemoglobin analysis shows mainly
HbBarts (4) with a small amount of HbH (4); HbA, A2 and F are absent.
28. The diagnosis is most likely to be:
1 A) Haemoglobin H disease
2 B) Hydrops fetalis
3 C) Homozygous sickle cell anaemia
4 D) Congenital methaemoglobulinaemia
Answer B
Reference: 4.04 LT7 Abnormalities of haemoglobin genes
29. Which of the following is correct? -thalassaemia:
1 A) Results most commonly from gene deletions
2 B) Results most commonly from point mutations

3 C) Can result from an acquired gene deletion
4 D) Is an X linked recessive disorder
Answer A
Reference: 4.04 LT7 Abnormalities of haemoglobin genes
30. In the developed world, most patients who have homozygous or compound heterozygous forms
of -thalassaemia die as a result of:
1 A) Anaemia
2 B) Infections, including HIV, hepatitis C and bacterial infections
3 C) Transfusion-related iron overload
4 D) Bone marrow failure
Answer C
Reference: 4.04 Lec 1 Thalassaemia
31. Which of the following conditions does NOT present with a microcytic hypochromic anaemia?
1 A) Sideroblastic anaemia
2 B) -thalassaemia trait
3 C) -thalassaemia major
4 D) Hereditary spherocytosis
5
Answer D
Reference: 4.04 BCS1 Interpreting lab tests in anaemia
32. When fully constituted with iron and porphyrins, what is the maximal number of molecules of
oxygen that can be carried by one molecule of haemoglobin?
1 A) None
2 B) One
3 C) Two
4 D) Four
Answer D
The molecular structure of hemoglobin is 22. Each subunit has one oxygen carrying heme group.
Reference: Structure of normal Haemoglobin 4.04 LT 6

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C) Restriction fragment length polymorphism analysis

The deficiency of factor VIII causes prolongation of the APTT

D) Erythroid hyperplasia in the bone marrow

Curriculum Reference: 4.05, Theme Session 1 (Coronary artery disease)

You ask Mr Worthington some more questions.

Erythropoietin levels are increased in haemolytic anaemias.

B) Vitamin Bi2 is absorbed in the upper small bowel

Mrs van Hilst's Results

Curriculum Reference: 4.01 Lecture 4: Iron deficiency

D) Impaired oxygen carrying ability

A) The cardiac output is reduced

58. What is the best interpretation of these results?

50. With respect to splenomegaly, which one of the following is correct?

Increased red cell destruction contributes to the associated anaemia

Bone marrow infiltration

3.5 % (normal range 1.5 - 3.5%)

54. Which of the following is correct concerning haemoglobin?

It features an oxygen binding, copper-chelated porphyrin-ring functional group

Excess production of a structurally normal -globin chain

Excess production of a structurally abnormal -globin chain

A full blood count on fetal blood

The placenta produces inhibitors of maternal red cell production

Iron deficiency due to heavy periods

Re-utilization of iron from phagocytosed red cells prevents iron deficiency

Author: Julie Curtin Dept of Haematology, Childrens Hospital at Westmead.

Author: Bill Webster, Anatomy Curriculum

27. Which one of the following is most correct?

3.5 % (normal range 1.5 - 3.5%)

2 B) Results most commonly from point mutations

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