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Hemat Q

A 78-year-old lady is admitted for surgery to remove part of her colon due to cancer. Post-operatively, she develops jaundice and fatigue. Investigations show a decrease in hemoglobin and increase in MCV. The direct Coombs test would help confirm the diagnosis of a blood transfusion reaction.

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100% found this document useful (1 vote)
227 views25 pages

Hemat Q

A 78-year-old lady is admitted for surgery to remove part of her colon due to cancer. Post-operatively, she develops jaundice and fatigue. Investigations show a decrease in hemoglobin and increase in MCV. The direct Coombs test would help confirm the diagnosis of a blood transfusion reaction.

Uploaded by

aliaa
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as DOCX, PDF, TXT or read online on Scribd
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1-A 78-year-old lady is admitted to hospital for a right

hemicolectomy for carcinoma of


the caecum.
She has a history of osteoarthritis for which she takes non-steroidal
anti-inflammatory
agents intermittently. Two years previously she had a fibroma
removed from her right
breast. She is a non-smoker and drinks approximately 8 units of
alcohol per week.
Investigations pre-operatively show:
Hb 105 g/L (115-165)
MCV 71 fL (80-96)
WCC 8.4 ×109/L (4-11)
Platelets 401 ×109/L (150-400)
The procedure was uncomplicated and she was given two units of
packed red cells
postoperatively. Three days later she becomes jaundiced and
complains of lassitude.
Investigations post-operatively:
Hb 72 g/L (115-165)
MCV 110 fL (80-96)
WCC 9.5 ×109/L (4-11)
Platelets 395 ×109/L (150-400)

Which of the following is the best investigation to confirm the


diagnosis?

A Antiglobulin test (indirect)


B- Direct Coombs' test
C- Haptoglobin level
D- Reticulocyte count
E- Serum iron level

2-A 32-year-old Afro-Caribbean lady is admitted to hospital with a


three day history of
increasing pain and swelling of her left leg. Over the past 24 hours
she has also
developed a low-grade fever. There is no history of recent trauma,
immobilisation or
prolonged travel by land or air.
She was admitted two years previously with a similar episode of
pain and swelling in her
right calf; a deep vein thrombosis was confirmed on Doppler
scanning. She is otherwise
fit and well. She is married and lives with her husband and works
on a production line
for a company producing televisions. She is a non-smoker and does
not drink alcohol.
She had been taking the oral contraceptive pill when admitted two
years ago with a
DVT, but this was then discontinued. Her only medication is an
antidepressant,
prescribed by her GP after she had a miscarriage six months
previously. Her
relationship with a previous partner ended after she miscarried his
child.

Investigations showed:
Hb 105 g/L (115-165)
MCV 94 fL (80-96)
WCC 7.5 ×109/L (4-11)
Platelets 95 ×109/L (150-400)
Protein C Normal activity -
Protein S Normal activity -
VDRL Positive 1:8 –

What is the likely diagnosis?


A- Antiphospholipid syndrome
B- Factor V Leiden mutation
C- Hyperhomocysteinaemia
D- Pelvic inflammatory disease
E- Tertiary syphilis

3-A 25-year-old woman undergoing treatment for promyelocytic


(M3) leukaemia
develops a high fever.
Sepsis is suspected and she rapidly develops widespread ecchymoses
and starts
bleeding from venepuncture sites and mucous membranes.
Which is the most specific laboratory test to confirm the diagnosis of
disseminated
intravascular coagulation (DIC)?

A- Activated partial thromoplastin time


B- D-dimer assay and fibrin degradation products (FDP)
C- INR
D- Plasma fibrinogen concentration
E- Platelet count

4-A 28-year-old lady presents with a three day history of a painful


swollen right calf.
Her coagulation screen shows:
Prothrombin time 13 s (11.5-15.5)
Thrombin time 13 s (13)
Activated partial thromboplastin time 78 s (30-40)
The APTT was not corrected when mixed with normal plasma.
What is the cause of the clotting abnormality?

A- Chronic liver disease


B- Disseminated intravascular coagulation
C- Haemophilia
D- Lupus anticoagulant
E- von Willebrand disease

5-A 72-year-old man is referred to clinic with a four month history


of breathlessness,
abdominal swelling and discomfort.
On examination, he appears pale and unwell. His liver is palpable 9
cm below the right
costal margin and his spleen palpable 7 cm below the left costal
margin. No
lymphadenopathy was detected.
Investigations show:
Hb 79 g/L (130-180)
RBC 2.1 ×1012/L -
PCV 0.17 -
MCH 30 pg (28-32)
MCV 82 fL (80-96)
Reticulocytes 1.4% (0.5-2.4)
Total WBC 23 ×109/L (4-11)
Normoblasts 8% -
Platelets 280 ×109/L (150-400)
Neutrophils 9.0 ×109/L (1.5-7)
Lymphocytes 5.2 ×109/L (1.5-4)
Monocytes 1.3 ×109/L (0-0.8)
Eosinophils 0.2 ×109/L (0.04-0.4)
Basophils 0.2 ×109/L (0-0.1)
Metamyelocytes 5.1 ×109/L -
Myelocytes 1.6 ×109/L -
Blast cells 0.4 ×109/L -
Blood film: Anisocytosis, poikilocytosis and occasional erythrocyte
tear drop cells.
What is the first investigation of choice?
A- Bone marrow culture for mycobacteria
B- Bone marrow smear
C- Bone scan
D- Bone (trephine) biopsy
E- Leucocyte alkaline phosphatase

6- A 33-year-old female with a history of paranoid delusions of one


month duration was
treated with phenothiazine.
She presents with a 10 day history of fever, chills, and malaise, with
increasing
weakness and fatigue over the last two days.
Examination reveals a temperature of 38.1°C, a pulse of 100 beats
per minute regular
and a blood pressure of 110/76 mmHg. She has a respiratory rate of
25/min and chest
with dullness to percussion and decreased breath sounds at the left
base. There is no
splenomegaly.
Investigations reveal:
Haemoglobin 102 g/L (115-165)
Haematocrit 0.384 (0.36-0.47)
MCV 90 fL (80-96)
White cell count 0.9 ×109/L (4-11)
Neutrophils 0.3 ×109/L (1.5-7)
Lymphocytes 0.3 ×109/L (1.5-4)
Monocytes 0.01 ×109/L (0-0.8)
Eosinophils 0.01 ×109/L (0.04-0.4)
Platelets 210 ×109/L (150-400)
Serum sodium 131 mmol/L (137-144)
Serum potassium 3.3 mmol/L (3.5-4.9)
Serum urea 4.2 mmol/L (2.5-7.5)
Serum glucose 5.1 mmol/L (3.0-6.0)
The chest x ray shows evidence of left basal consolidation.
What is the most likely cause of her haematological indices?

A- Accelerated destruction of neutrophils


B- Aplastic anaemia
C- Drug induced
D- Ineffective erythropoiesis
E- Shift from circulating pool to marginal granulocyte pool

7- A 27-year-old Afro-Caribbean male attends for a routine


insurance examination.
Questioning reveals that he has been frequently tired of late and
sleeps in the evenings.
He also gives a history of bleeding gums and several recent episodes
of epitaxis. He is
a non-smoker but drinks 22 units of alcohol weekly.
Examination reveals a pale appearance but no other abnormalities.
Investigations show:
Haemoglobin 80 g/L (130-180)
Haematocrit 0.24 (0.40-0.52)
MCV 88 fL (80-96)
White cell count 2 ×109/L (4-11)
Neutrophils 20% -
Lymphocytes 77% -
Platelets 40 ×109/L (150-400)
The peripheral blood film showed normocytic hypochromic
erythrocytes.
What is the most likely diagnosis?

A- Accelerated destruction of neutrophils


B- Aplastic anaemia
C- Drug induced
D- Sepsis
E- Sickle cell crisis

8- A 55-year-old man was referred to the outpatient clinic with


anaemia.
He had presented to his general practitioner with a three month
history of fatigue and
low back pain. There was no preceding history of trauma. A plain x
ray of his lumbar
spine showed a lytic lesion in the body of the fourth lumbar vertebra
(L4).
Investigations revealed:
Haemoglobin 105 g/L (130-180)
WBC 4.0 ×109/L (4-11)
Platelets 175 ×109/L (150-400)
Serum sodium 137 mmol/L (137-144)
Serum potassium 3.5 mmol/L (3.5-4.9)
Serum urea 2.7 mmol/L (2.5-7.5)
Serum creatinine 110 µmol/L (60-110)
Serum corrected calcium 2.4 mmol/L (2.2-2.6)
Bence Jones proteins were detected in the urine.
A skeletal survey showed increased uptake in the L4.

Which of the following therapies will reduce the risk of pathological


fracture?

A- Autologous stem cell transplantation


B- Melphalan
C- Pamidronate
D- Plasmapheresis
E- Steroids
9- A 32-year-old Nigerian lady with sickle cell anaemia (Hb SS) has
a history of
recurrent back pain.
She presents to casualty with fever and a worsening of the back
pain. There is no
history of weight loss or night sweats.
Investigations show:
Haemoglobin 78 g/L (115-165)
White cell count 10.1 ×109/L (4-11)
Platelets 475 ×109/L (150-400)
Reticulocytes 12%
Serum total bilirubin 88 µmol/L (1-22)
What is the most likely diagnosis?

A- Aplastic crisis
B- Haemolytic crisis
C- Malaria
D- Tuberculosis
E- Vasoocclusive event

10 - A 26-year-old man attends the dental hospital for a tooth


extraction.
He reports prolonged bleeding after a tooth extraction two years
previously. He has had
no other surgical challenges and no other history of prolonged
bleeding. He is otherwise
fit and well.
On further questioning he said his mother had recently been
referred to a local hospital
for recurrent nose bleeds and menorrhagia. He was also aware that
his sister had been
seen in the past for menorrhagia.
The dental extraction cannot be delayed.
What should the dentist be advised to give the patient to reduce the
risk of significant
bleeding?
A-Cryoprecipitate
B- Factor VIII replacement
C- Fresh frozen plasma (FFP)
D- Tranexamic acid
E- von Willebrand factor concentrate

11- A 56-year-old man with chronic renal failure attended the renal
clinic.
He was undergoing regular haemodialysis and had been treated for
the past six months
with oral ferrous sulphate (200 mg three times a day). His
haemoglobin in clinic
measured at 76 g/L, compared with 106 g/L six months previously.
Which of the following is the most appropriate treatment?

A- Erythropoietin
B- Increase the dose of oral ferrous sulphate
C- Intravenous iron
D- Intravenous iron and subcutaneous erythropoietin
E- Transfusion of two units of packed red cells

12- A 64-year-old lady is reviewed in the outpatient clinic. She has


been known to have
chronic lymphocytic leukaemia (CLL) for six months.
Apart from three chest infections in the last year, she is otherwise
well.
Investigations show:
Haemoglobin 134 g/L (115-165)
White cell count 30.2 ×109/L (4-11)
Lymphocytes 26.2 ×109/L (1.5-4)
Neutrophils 3.8 ×109/L (1.5-7)
Platelet count 350 ×109/L (150-400)
Serum electrophoresis:
IgG 2.5 g/L (6-13)
IgA 0.2 g/L (0.8-3.0)
IgM 0.1 g/L (0.4-2.5)
What is the most appropriate management option at this time?

A- Chlorambucil
B- Fludarabine
C- Intravenous immunoglobulin infusions
D- Observe
E- Stem cell transplant

13- A 15-year-old girl is referred urgently to clinic because of a two


day history of
spontaneous bruising.
She has no past history of note and is not taking any regular
prescribed medication.
She has noticed spontaneous appearance of bruises on her hips,
thighs and upper
arms over the past three days. There is no history of trauma to
account for their
appearance. The largest of these measures 15 cm in diameter.
Otherwise she feels
well, though she reports having had a mild viral illness two weeks
previously.
Investigations show:
Haemoglobin 141 g/L (115-165)
White blood cells 7.3 ×109/L (4-11)
Platelets 15 ×109/L (150-400)
What is the most important next step?
A- Blood film examination
B- Bone marrow biopsy
C- Check coagulation screen.
D- Reassure that this is likely to resolve and see again in five days
E- Start prednisolone treatment

14- A 34-year-old man presents with a swollen, painful right calf.


A Doppler ultrasound confirms the presence of a posterior knee
occlusive deep vein
thrombosis (DVT). Two weeks previously a below knee plaster cast
had been removed
on the right side. This had been in place for four weeks following a
rugby injury. He had
no other significant past medical history.
What is the best management plan for this man?

A- Low molecular weight heparin followed by warfarin for six months


B- Low molecular weight heparin followed by warfarin for three
months.
C- No treatment necessary
D- TED support stockings and repeat Doppler in one week.
E- Warfarin only
15- An 18-year-old Zimbabwean man presented with a two week
history of massive
painless cervical lymphadenopathy.
Which of the following investigations should be performed to obtain
the diagnosis?

A- Bone marrow aspiration and trephine biopsy


B- Chest x ray
C- CT scan chest
D- HIV antibody test
E- Lymph node biopsy

16- A 34-year-old man was admitted to hospital with a painful


swollen left leg.
While in the admissions unit he also developed abdominal pain -
which he said had
been recurring over several months. He had a past history of a right
calf deep vein
thrombosis (DVT) six months previously that had been treated by
anticoagulation with
warfarin for three months. He had noticed that over the past two
months his urine had
been darker than usual in the morning.
Investigations showed:
Haemoglobin 85 g/L (130-180)
White cell count 2.5 ×109/L (4-11)
Platelets 75 ×109/L (150-400)
PT 12 seconds (11.5-15.5)
APTT 35 seconds (30-40)
Serum total bilirubin 29 µmol/L (1-22)
Serum AST 20 U/L (1-31)
Serum alkaline phosphatase 80 U/L (45-105)
What is the most likely diagnosis?
A- Acute intermittent porphyria
B- Autoimmune haemolytic anaemia
C- Factor V Leiden mutation
D- Paroxysmal nocturnal haemoglobinuria
E- Protein C deficiency

17- A 33-year-old male develops a rash and low grade fever (37.6°C)
21 days post
allogeneic bone marrow transplant for high risk acute myeloid
leukaemia in first
complete remission.
The rash is initially maculopapular affecting palms and soles but 24
hours later general
erythroderma is noted affecting the trunk and limbs. His total
bilirubin was previously
normal but is now noted to be 40 µmol/L (1-22). He remains very
well in himself.
What would be your management of this patient at this stage?

A-Antibiotics after blood cultures


B- Antilymphocyte globulin
C- High dose methylprednisolone
D- Observation
E- Prednisolone

18- A 69-year-old lady is seen in the haematology clinic for the


second time. Her first
review was twelve months previously.
Her full blood count shows:
Haemoglobin 118 g/L (115 - 165)
White cell count 79 ×109/L (4 - 11)
Neutrophils 4 ×109/L (1.5 - 7)
Lymphocytes 74.5 ×109/L (1.5 - 4)
Monocytes 0.4 ×109/L (0 - 0.8)
Eosinophils 0.05 ×109/L (0.04 - 0.4)
Basophils 0.05 ×109/L (0 - 0.1)
Platelet count 385 ×109/L (150 - 400)
Her white cell count twelve months previously had been 50 ×109/L.
She is very well.
How do you manage her at this stage?

A- Chlorambucil
B- Fludarabine
C- Leucapharesis
D- Observation
E- Prednisolone

19- A 33-year-old lady is admitted to the gynaecology ward at 24


weeks gestation in her
first pregnancy.
She had seen her midwife and complained of feeling generally not
well. Her husband
reported that she has become very vague and disorientated. The
midwife found her
blood pressure to be 140/89 mmHg; her booking blood pressure was
120/80 mmHg.
On admission her blood tests are as follows:
Haemoglobin 79 g/L (115-165)
White cell count 11 ×109/L (4-11) Normal differential
Platelets 30 ×109/L (150-400)
MCV 103 fL (80-96)
Reticulocyte count 12.5% -
Blood film shows red cell fragmentation with thrombocytopenia and
platelet
anisocytosis. There is polychromasia.
Serum lactate dehyrogenase 1112 U/L (10-250)
Serum sodium 140 mmol/L (137-144)
Serum potassium 3.7 mmol/L (3.5-4.9)
Serum urea 15.2 mmol/L (2.5-7.5)
Serum creatinine 200 µmol/L (60-110)
Serum total bilirubin 73 µmol/L (1-22)
Serum alanine aminotranseferase 40 U/L (5-35)
Serum alkaline phosphatase 160 U/L (45-105)
Coagulation screen Normal -
Fibrinogen 5.3 g/L (1.8-5.4)
What treatment would you advise the obstetric team to give to this
lady?

A- Fresh frozen plasma transfusion


B- Haemodialysis
C- None
D- Plasma exchange
E- Platelet transfusion

20- An 18-year-old man was admitted to casualty at 3 am having


collapsed at a rave
party.
A friend, who accompanied him to hospital, said that the patient
had been entirely well
earlier in the evening and had been dancing most of the night. He
reported that his
friend seldom drank alcohol, although there was alcohol available at
the party venue.
He did not know whether his friend had taken any drugs.
Investigations revealed:
Haemoglobin 89 g/L (130 - 180)
White cell count 25.0 ×109/L (4 - 11)
Neutrophils 21.7 ×109/L (1.5 - 7.0)
Lymphocytes 2.0 ×109/L (1.5 - 4.0)
Monocytes 0.8 ×109/L (0 - 0.8)
Eosinophils 0.4 ×109/L (0.04 - 0.4)
Basophils 0.1 ×109/L (0 - 0.1)
Platelets 27 ×109/L (150 - 400)
Reticulocyte count 10%
Prothrombin time 32 secs (11.5 - 15.5)
Activated partial thromboplastin time 82 secs (30 - 40)
Fibrinogen 0.3 g/L (1.8 - 5.4)
D-Dimer screen 2.6 mg/L (<0.5)
Serum sodium 137 mmol/L (137 - 144)
Serum potassium 6.3 mmol/L (3.5 - 4.9)
Serum urea 17.5 mmol/L (2.5 - 7.5)
Serum creatinine 200 µmol/L (60 - 110)
Lactate dehydrogenase 525 U/L (10 - 250)
A blood film showed red cell fragmentation with polychromasia,
toxic granulation of
neutrophils and platelet anisocytosis.
What is the diagnosis?

A- Autoimmune haemolysis
B- Autoimmune thrombocytopenia
C-Disseminated intravascular coagulation (DIC)
D- Drug-induced haemolysis
E- Thrombotic thrombocytopenic purpura (TTP)

21- A 53-year-old gentleman was admitted to hospital after


presenting to his GP
complaining of feeling lethargic and generally unwell.
Despite having a normal appetite, he had lost two stones in weight in
six weeks. He also
reported sweating profusely at night and his wife said that she often
had to change the
sheets because they were drenched. He also has some upper
abdominal discomfort.
On examination he looked thin and unwell. His pulse was 90 beats
per minute in sinus
rhythm with blood pressure 145/80 mmHg. A short systolic murmur
was audible at the
lower left sternal edge. His chest was clear. His abdomen was soft,
with slight
tenderness in the epigastrium and central abdomen. The spleen was
palpable 7 cm
below the left costal margin.
Investigations revealed:
Haemoglobin 97 g/L (130-180)
White cell count 17.4 ×109/L (4-11)
Neutrophils 14.0 ×109/L (1.5-7)
Lymphocytes 1.5 ×109/L (1.5-4)
Monocytes 0.8 ×109/L (0-0.8)
Basophils 0.7 ×109/L (0-0.1)
Eosinohils 0.4 ×109/L (0.04-0.4)
Platelets 550 ×109/L (150-400)
The blood film showed left-shifted neutrophils with numerous
myelocytes present.
Occasional promyelocytes were seen, but no blasts. There were also
a number of
nucleated red blood cells as well as a large number of tear drop red
cells. There was a
thrombocytosis with platelet anisocytosis.
What investigation should be performed next?

A- Cytogenetic analysis of the patient’s bone marrow


B- Immunophenotyping of peripheral blood
C- Lactate dehydrogenase
D- Neutrophil Alkaline Phosphatase score
E- Ultrasound scan of abdomen
22- A 62-year-old man attended the outpatient clinic for a follow up
appointment. He had
a history of two venous thromboembolic events and wanted advice
regarding his
treatment with warfarin.
He had been diagnosed with an ilio-femoral deep vein thrombosis
two years previously
and had been treated with a six month course of warfarin. A
thrombophilia screen,
performed two months after stopping the warfarin, was negative.
Six months after stopping warfarin he presented to hospital with
left-sided pleuritic chest
pain. His ECG was normal but a CT pulmonary angiogram showed
a pulmonary
embolus. On this occasion there were no obvious risk factors, other
than his previous
event. He was recommenced on warfarin.
At the time of his follow up outpatient appointment he was
approaching completion of
six months of treatment. During his hospital admission, he had been
advised that he
should receive lifelong treatment with warfarin. However, he
expressed concern about
the risk of bleeding while on warfarin.
On further questioning, he reveals that he has had two admissions to
hospital with
episodes of bleeding in the past three months. On the first occasion
he had a
spontaneous epistaxis. The second admission was for bleeding from
a scalp wound
after he hit his head accidentally on the bathroom cupboard. On
both occasions his INR
was over 8 and he had been admitted until the INR returned to
normal.
What is the best course of action?
A- Continue warfarin and check compliance and monitoring of INR
B- Stop warfain
C-Stop warfarin and give long term low molecular weight heparin
D- Stop warfarin and implant an inferior vena caval filter
E- Stop warfarin and receive low molecular weight heparin for long
journeys

23- A 33-year-old woman developed a fever (38.0°C) within 90


minutes of starting a red
cell transfusion three days post allogeneic bone marrow transplant.
Prior to the
transfusion her temperature had been 37.6°C.
On examination she appeared flushed, breathless and complained of
dizziness and a
tight chest. Her heart rate was 110 beats per minute and regular,
blood pressure was
90/60 mmHg and auscultation of the chest revealed widespread
wheeze.
The staff nurse stopped the transfusion and checked a full blood
count, the result of
which is shown below:
Haemoglobin 88 g/L (115-165)
White cell count 0.8 ×109/L (4-11)
Neutrophils 0.0 ×109/L (1.5-7)
Lymphocytes 0.5 ×109/L (1.5-4)
Monocytes 0.2 ×109/L (0-0.8)
Eosinophils 0.1 ×109/L (0.04-0.4)
Basophils 0.0 ×109/L (0-0.1)
Platelet count 12 ×109/L (150-400)
What other action should be taken?

A- Ask the blood bank for a further cross match of leucodepleted blood
B- Give paracetamol and keep close observation
C- Give paracetamol and restart transfusion at a slower rate
D- Give paracetamol, hydrocortisone and chlopheniramine
E-Start intravenous antibiotics to cover possible hospital acquired
pneumonia

24- A 55-year-old gentleman presented to the outpatient clinic after


being referred by his
general practitioner.
He complained of feeling lethargic and had lost 15 kg in weight. He
also complained of
profuse sweating, especially at night and also had some upper
abdominal discomfort.
On examination, the spleen was palpable 12 cm below the left costal
margin.
Investigations revealed:
Haemoglobin 97 g/L (130-180)
White cell count 17.4 ×109/L (4-11)
Neutrophils 14.0 ×109/L (1.5-7)
Lymphocytes 1.5 ×109/L (1.5-4)
Monocytes 0.8 ×109/L (0-0.8)
Basophils 0.7 ×109/L (0-0.1)
Eosinohils 0.4 ×109/L (0.04-0.4)
Platelets 550 ×109/L (150-400)
The blood film was reported as follows: The neutrophils are left
shifted with numerous
myelocytes present. There is an occasional promyelocyte but no
blasts. There are also
a number of nucleated red blood cells. There is a thrombocytosis
with platelet
anisocytosis.
What investigation should be performed next?
A- Immunophenotyping of peripheral blood
B- Lactate dehydrogenase (LDH) measurement
C- Molecular analysis of peripheral blood
D- Neutrophil alkaline phosphatase (NAP) score
E- Ultra sound scan (USS) of abdomen

25- What is true about iron absorption and metabolism?

A- High levels of hepcidin are necessary for proper iron absorption


B- Heme iron is absorbed by the same intestinal receptor as nonheme
iron
C- Dietary iron is present mostly in the ferrous form and must be
oxidized for absorbtion
D- Hepcidin's mechanism of action is by degradation of ferroportin,
therefore decreasing iron absorbtion
E- IL6 upregulates hepcidin production and high IL6 levels
underlies the refractory anemia in some cases of Castleman's
disease.

26 - What is true about RBC exchange in sickle cell disease?


A- It has been proven to improve pregnancy outcome when used
chronically
B- Should be performed prior to any surgical procedure instead of
simple transfusion
C- Is effective only when it raises Hb level to over 12
D- May be difficult or impossible to perform in patients with
multiple alloantibodies
E- Requires an apheresis machine as it can not be performed manually.

27- What is true about the sickling process?

A-It takes place in the post capillary venules which are too large to
be occluded by single cells
B- Cytokines have no role in acceleration of sickling
C- Thrombocytosis is universal in sickle cell disease but does not play a
role in sickling as the platelets are not activated.
D- Leukocytosis is common in sickle cell disease but does not play a
role in sickling as the neutrophils are not activated.
E- Since RBC adhesion involves plasma proteins, plasma exchange
effectively halts sickling.

28- What is true about coagulation in sickle cell disease?

A- Ischemic stroke is common and predominantly involves the


microvessels
B- Loss of normal membrane phospholipids symmetry occurs due to a
mutation in the "flipase" enzyme, whose gene is present linked on
chromosome 11.
C- Aspirin is effective in preventing thrombosis because the
thrombocytosis is due to functional asplenia
D- The pathophysiology of hypercoagulability involves the interaction
of coagulation proteins with hepatocytes.
E- Nitric oxide depletion by hemolysisn which releases free
hemoglobinn causes vasoconstriction and contributes to the
tendency to clotting in many hemolytic diseases (sickle cell, PNH
etc).

29 - What is true about the anemia of beta-thalassemia major and


intermedia?

A- It is caused by relative hypoplasia of the RBC series in the marrow,


with low retic counts.
B- The degree of anemia is generally well correlated with the
severity of the mutations carried by the patient, so accurate genetic
counseling is possible.
C- Is exacerbated by concurrent alpha thalassemia and ameliorated by
excess alpha globin genes.
D- Is unaffected by mutations causing elevation of fetal hemoglobin
E- Can only be relieved by blood transfusions since no drugs (such as
erythropoietin, hydrea, and other agents) have ever been shown to be
effective.

30- A 62-year-old gentleman was followed up every six months in


the haematology clinic.
During his most recent appointment he complained of a headache,
blurred vision,
aching bones, and extreme lethargy. His wife reported that he has
been very vague at
times over the previous four weeks and drowsy when he was awake.
On examination he appeared pale with blood pressure 125/65
mmHg. He was afebrile.
Respiratory rate was 12 breaths per minute. Cardiovascular, chest
and abdominal
examination were unremarkable.
Further investigations showed:
Haemoglobin 82 g/L (130-180)
MCV 88 fL (80-96)
White cell count 9.0 ×109/L (4-11)
Neutrophils 6.2 ×109/L (1.5-7)
Lymphocytes 2.0 ×109/L (1.5-4)
Monocytes 0.6 ×109/L (0-0.8)
Eosinophils 0.1 ×109/L (0.04-0.4)
Basophils 0.1 ×109/L (0-0.1)
Platelet count 160 ×109/L (150-400)
Serum sodium 137 mmol/L (137-144)
Serum potassium 4.8 mmol/L (3.5-4.9)
Serum urea 9.0 mmol/L (2.5-7.5)
Serum creatinine 130 µmol/L (60-110)
Serum aspartate aminotransferase 30 U/L (1-31)
Serum alkaline phosphatase 90 U/L (45-105)
Serum total bilirubin 22 µmol/L (1-22)
Serum phosphate 1.2 mmol/L (0.8-1.4)
Serum corrected calcium 2.7 mmol/L (2.2-2.6)
Serum albumin 26 g/L (37-49)
Serum total protein 120 g/L (61-76)
Serum C-reactive protein 67 mg/L -
Serum immunoglobulins:
IgG 0.6 g/L (6.0-13.0)
IgA 78 g/L (0.8-3.0)
IgM 0.2 g/L (0.4-2.5)
Serum electrophoresis showed a monoclonal band (IgA kappa) with
immune paresis.
The IgA kappa level was 76 g/L.
Which of the following would be the best treatment option for this
gentleman?
A- Blood transfusion
B- Chemotherapy
C- Intravenous fluids and furosemide
D- Plasma exchange
E- Plasma exchange and chemotherapy

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