hematology PT 2016 modif

A 54-year-old man presents wit h a 2-mo nth history of increasing lethargy. He also reports
that he has b een bruising more easi ly of late. On d irect questioning he admits to p assing
dark urine, but denies any other urinary symptoms. A year ago he had finished a 6-mo nth
period of ant icoagulant therapy for a spontaneous deep vein t hrombosis. His full blood
count demo nstrates haemoglobin 6 .5 g/dl, white b lood-cell count 2.8 x 109/1, and platelets
27 x 109/1.
What is the most appropriate management for his anaemia?
A

Androgen 'hormonal' therapy

B

Erythropoietin injections

c

Folic acid therapy

D

Transfusion with blood substitutes

E

Transfusion with red cells


Explanation

(I

The answer is Transfusion wich red cells -

Transfusion with red cells is the best way to manage this man's anaemia. Essentially, this
man is pancytopenic. He has a background history of a deep vein thrombosis, and this
combinat ion should suggest a diagnosis of paroxysmal nocturnal haemoglobinuria
( PNH). This can also be associated with aplastic anaemia, and a bone marrow
examination should certainly be performed to d iagnose this. There is historical literature
to suggest that washed red cells was a superior transfusion product for PN H patients.
However, there is no evidence that survival of washed red cells is better than that of
standard red cells.

Androgen ' hormonal' therapy (Option A) is incorrect.
Androgen hormonal therapy again can be regarded as an adjunctive therapy, but would not
replace red cell transfusion.
Erythropoietin injections (Option B) is incorrect.
It would be expected that this man's erythropoietin levels will naturally be high as a result of
the anaemia, and therefore additional erythropoietin supplementation will not be benef icial.
Folic acid therapy (Option C) is incorrect.
Folic acid therapy is an important adjunct fo r all patients who are haemolysing, but cannot
be regarded as more appropria te than standard transfusion with red cells.
Transfusion with blood substitutes (Option D) is incorrect.
Transfusion with blood substitutes is certainly not a correct answer, as these are not
routinely available.


A 30 -year-old woman is admitted with a right-sided hemiparesis. She returned 2 days earlier
from visiting her sister in Australia. She has b een generally well ap art from occasional
migraine headaches. The o nly medication of note is the progestero ne-only contracep tive p il l.
On examination her BP is elevated at 152/ 89 mmHg and she has a right-sided hemiparesis.

You note that her right leg is swollen. Her flat mate tells you that she complained that the leg
was aching just after she returned home.
Investigatio ns:
Hb

124 g/ 1

w ee

6.1 x 10 9/ 1

PLT

210 x 10 9/ 1

Na+

140 mmo l/1

K+

4.9 mmo l/1

ereatinine 109 µmol/1
ESR

lOmm/ h

Which one of the fol lowing investigations would be most helpfu l in reveal ing the cause of
her stroke?

Which one of the following investigations would be most helpful in revealing the cause of
her stroke?
A

Abdominal ultrasound scan

B

Carotid d uplex

C

CT head

D

Echocardiography

E

Right leg v enogram


Explanation
The answer is Echocardiography It would appear that this lady has suffered a right leg deep vein thrombosis ( DVT), but
also has a right-sided hemiparesis. This would lead to the possibility of a paradoxical
embolus through a patent foramen ova le, causing 1,er stroke. This may well be id entified
by echocardiog raphy.

Abdominal ultrasound scan (Option A) is incorrect. Abdominal ultrasound scan will not help
in elucidating the cause of her stroke.
Carotid duplex (Option B) is incorrect. Carotid duplex would not tie in with her possible
right DVT.
CT head (Option C) is incorrect. CT head will determine whether or not she has had a
thrombotic orhaemorrhagic stroke, but will not be helpful in revealing the cause of her
stroke.
Right leg venogram (Option E) is incorrect. Right leg venogram wou ld nowadays be
superseded by ultrasonography. Although this might confirm a coincidental DVT,
demonstration of a patent foramen ovale will be most helpful in revealing the cause of her
stroke.


Which one of the following features is characteristic o f immune thrombocyt openic purpura
(ITP)?
A

Infants b orn to a wo man wi th ITP often p resent with bleeding d iathesis in the fi rst
48h

B

Pa ncyt openia is a recognised complication

c

Leukaemic transformation occurs late in the disease

D

Splenomegaly is found in 50% of cases

E

Autoimmune haemolytic anaemia is a recognised association


Explanation
The answer is autoimmune haemolycic anaemia is a recognised association When ITP co-exists with an autoimmune haemolytic anaemia, it is known as Evans
syndrome. The management of this condition is usua lly the same, namely wi th
immunosuppression using steroids as a firstline treatment.

Infants born to a woman with ITP often present with bleeding problems in the first 48 h
(Option A) is incorrect. Although the infant's platelet count may decrease after birth, this is
rarely severe enough to induce significant bleeding in the new-born infant.
Pancytopenia (Option B) is not a recognised complication of ITP.
Leukaemic transformation occurs late in the disease(Option C) is incorrect. There is no
evidence of increased leukaemic transformation in ITP.
Splenomegaly is found in 50% of cases(Option 0) is incorrect. Although the site of
destruction of the platelets is in the spleen, it is rare to get significant splenomegaly and
certainly not in 50% of cases.


A 49 -year-o ld wo man who is 12 months post-chemotherapy for carcinoma of the breast
comes to the clinic for review. The reg ime included use o f trastuzumab. She has significant
shortness of breath which has increased over the past 4 weeks. On examination her BP is
135/ 80 mmHg, pulse 92 beats/min regular. Her JVP is raised 6 cm and she has bilateral ankl e
swelling. Respiratory examination revealed bibasal crackles.
~Vhich one of the following is the most likely diagnosis?

A

Chemotherapy-related pulmonary fibrosis

B

Radiotherapy-related pulmonary fibrosis

C

Malignam pleural effusion

D

Lymphangitis carcinomatosis

E

Chemotherapy-related cardiac failure


Explanation
Adverse effects of cancer treatment
ERB-B2 expression may be crucial in preventing the development of di lative
cardiomyopathy; hence use of an inhibitor such as trastuzumab is likely to result in
increased risk of cardiac failure
vVhilst it would seem somewhat surprising that this wo man presented 12 months after
chemotherapy, one study showed that patients with early signs of cardiomyopathy
developed worsening symptoms of heart failure over time
If the JVP wasn't raised then lymphangit is/pulmonary infi ltration would have been a

consideration
(Journal of Clinical Oncology 2007; Vo lume 25 Number 23)


A 56-yea r-old gentleman with relapsed CLL is treated with fludarabine. The ful l blood count
is haemoglobin 5 g/1, MCV 120 fl, white blood count 140 x 109/1 and platelets 110 x 109/1. As
he is symptomatic a 3-unit blood transfusion is administered. Four days later he presents
with increasing shortness of breath. The P02 is 89% on air and a chest X-ray shows
shadowing at both hila.
Which one of the following is the most likely diagnosis?
A

Alveolar haemorrhage

B

Atypical pneumonia

C

Pneumocystis jirovecii infection

D

Pul mo nary oedema

E

Transfusio n-associated g raft -versus-host disease

Explanation
The answer is Pneumocyscis jirovecii infection This man has been treated with a purineanalogue in the form of fludarabine. This
cytotoxic agent affects T-cell function. Patients aretherefore prone to opportunistic
infections includi ng pneumocystis infection. Patients therefore receiving pur ine
analogues should also receive co-trimoxazole to red uce this r isk.

Alveolar haemorrhage (Option A) is incorrect. The p latelet count is satisfactory at 110 and
there is nothingin particular to suggest this d iag nosis.
Atypical pneumonia (Option B) is incorrect. Atypical pneumonia could be a possibility, but
pneumocystis infection in the setting of fludarabine treatment is more common, particularly
with the chest X-ray find ings showing b i-hilar shadowing.
Pulmonary oedema (Option D) is incorrect. The chest X-ray appearances are not classical for
this.
Transfusion-associated graft-versus-host disease (Option E) is incorrect. All patients who
receive purineanalogues are at risk of transfusion -associated graft-versus-host d isease and
therefore shouldreceive irradiated blood products. The clinical featu res of transfusionassociated graft-versus-host disease are pancytopaenia, liver dysfunction, diarrhoea and
rash, which is not the scenariothat is presented in this question.


Which malignancy is cu rrently the number one cause of death in women in the United
Kingdom?
A

Breast cancer

8

Colon cancer

c

Leukaemia

D

Lung cancer

E

Uterine cancer


Explanation

(I

The answer is Lung cancer Cancer is the cause of 26% of deaths in the UK, and is a more common cause of death
than cardiovascu lar disease. Lung cancer, with its low survival rates, is the biggest
cancer k iller in the UK, although b reast cancer has the highest incidence.In 2012 over
one-fifth (21%) of all cancer deaths in women were from lung cancer, 15% from breast
cancer, 9% from colon cancer. Leukaemia and uterine cancer are rarer, each accounting
for approximately 3% of cancer deaths in women.
In males the most common cause of cancer death is also Jung cancer (23%), with
p rostate (13%) and colon cancer (10%) also causing significant mortality.
On average 92 people die every day from lung cancer in the UK.Cigarette smoking has
been identified as the single most important cause of preventable disease and
p remature death in the UK.Overal l, one-third of all cancer deaths are Jinked to smoking.

[Ref: Cancer Research UK]

Breast cancer (Option A) is incorrect. Although, as describ ed, breast cancer has th e highest
incidence, it accounts for 15% of cancer deaths in women compared to 26% for lung cancer.
Colon cancer (Option B) is incorrect. Colon cancer accounted for approximately 9% of
cancer deaths in women.
Leukaemia (Option C) is incorrect. Leukaemia accounted for approximately 3% of cancer
deaths in women.
Uterine cancer (Option E) is incorrect. Uterine cancer accounted for ap proximately 3% of
cancer deaths in women.


Which one of the following statements is true about folic acid deficiency?
A

Because of the high folate body stores, it will take more than 2 years for
megaloblastic anaemia to develop after complete cessation of folic acid intake

8

Methotrexate-induced folic acid deficiency is corrected by concomitant folic acid
therapy

C

Intestinal bacterial overgrowth is regarded as one of the common causes

D

Causes abnormal neurological findings indistinguishable from that associated with
812 deficiency

E

Responsible for neural tube defect in the fetus


Explanation
The answer is Folic acid is responsible for neural tube defect in the fetus Folic acid is certainly responsible for neural tube defects in the fetus, and it is
recommended t hat pregnant women take supplementary folic acid in the early stages of
pregnancy.

Because of the high fola te body stores, it wil l take more than 2 years for megaloblastic
anaemia to develop after complete cessation of folic acid intake (Option A) is incorrect. It
does not take a long time to become folate deplete, because of the limi ted amount of folate
the body stores. This is therefore quite common in elderly patients with a poor diet.
Methotrexate-induced folic acid deficiency (Option B) is incorrect. Folinic acid rather than
fo lic acid effectively counteracts the action of methotrexate by by-passing the inhibited
reductase and is useful in the treatment of methotrexate toxicity.
Intestinal bacterial overgrow th (Option C) is not a common cause of folic acid deficiency;
this can cause 8 12 deficiency.
Causes abnormal neurologica l findings indistinguishable from that associated with 8 12
deficiency (Option D) is incorrect. The lack of neurolog ical find ings in patients with
megaloblastic anaemia suggests folic acid rather than 812 deficiency.


A 72-year-old woman presents with worsening back pain. She also feels generally weak. An
X-ray of her back shows multiple vertebral collapses and Jytic lesions. In addition she has had
an ep isode o f shingles over the last 6 months.
What is the next investigation to confirm the most likely d iagnosis?
A

Bone biopsy

B

CT scan

c

Magnetic resona nce imaging

D

Muscle biopsy

E

Serum electrophoresis


Explanation
The answer is Serum electrophoresis The next investigation that would likely confirm the d iagnosis is the one that can be
most easi ly performed. lmmunoglobulin levels and serum electrophoresis would be the
next investigation here. This is the most easi ly performed investigation.

Bone biopsy (Option A) is incorrect. Bone biopsy is a very invasive test that is not usually
required. A bone marrow examination, however, would be an important investigation - but
evaluation of immunoglobulin levels, identification of a paraprotein through serum
electrophoresis and examining serum free light chains would be more importan t.
CT scan (Option B) is incorrect. A CT scan is not going to be helpful here. A CT scan can be

important in helping to evaluate soft tissue plasmacytomas, but in general, MRI scanning is
more useful. It should be noted, however, that neither of these imaging modalities will
answer the question and confirm the most likely diagnosis of myeloma.
Magnetic resonance imaging (MRI) (Option C) is incorrect. MRI is going to be important to
make sure that this lady does not have any evidence of cord compression. It will also
delineate the extent of bony disease in much more detail. However, it is not going to help
confirm the likely diagnosis of myeloma.
Muscle biopsy (Option D) is incorrect. Muscle biopsy has got nothing to do with the most
likely d iagnosis of myeloma.


Which one of the following is the most likely finding in the stable phase of chronic myeloid
leukaemia?
A

Enlargement of t he spleen

B

Enlargement of lymph nodes

c

Pulmo nary infiltrates

D

Joint swelling

E

Enlargement of t he liver


Explanation
The answer is Enlargement of the spleen Chronic myeloid leukaemia, along with myelofibrosis, are the t wo major causes of
significant splenomegaly. It should be noted that splenomegaly is not always found in all
cases of chronic myeloid leukaemia. Options B, C, D and E are false.

Enlargement of lymph nodes (Option B) is incorrect, this is not the most likely finding in the
stable phase of chronic myeloid leukaemia.
Pulmonary infiltrates (Option C) is incorrect, this is not the most likely finding in the stable
phase of chronic myeloid leukaemia.
Joint swelling (Option D) is incorrect, this is not the most likely finding in the stable phase of
chronic rnyeloid leukaemia.
Enlargement of the liver (Option E) is incorrect, this is not the most likely finding in the
stable phase of chronic rnyeloid leukaemia.


A 24-year-old Kenyan student presents to the Emergency Department with fevers and
rigors. On examination he is found to have massive splenomegaly. He admits to having had
malaria in t he past and to having taken prop hyla xis intermittently. The laboratory staff
reports infection with Ptasmodium malariae after examining the b lood film. Antimalarial
treatment is commenced but 2 days later he is acutely unwell. The haemoglobin is 4.6 g/ 1,
reticulocy tes 40%, creatinine 450 mmol/1and there is o liguria.
What is the likely diagnosis?
A

Blackwater fever

8

Drug-induced haemolysis

c

G6PD deficiency

D

Sickle cell disorder

E

Tropical splenomegaly syndrome


Explanation
The answer is Blackwater fever This man has become very unwell after treatment fo r malaria has started. He has
b ecome acutely anaem ic. The raised reticulocyte count suggests either b leeding or
haemolysis. There is evidence o f acute renal impairment. This is consistent with the
develop ment of blackwater fever. This is a rare complicationconsisting of intravascu lar
haemolysis that occu rs in the acu te phase of malaria treatment. This can cause intense
jaundice, and can be associated with renal shutdown.

Drug- induced haemo lysis (Option B) is incorrect. Dr ug-i nduced haemolytic anaemia can
occur, b ut usually causes extravascularhaemo lysis. This man is very unwel l, suggesting
intravascular breakdown of haemoglobin with the develop ment of acute renal impairment.
G6PD deficiency ( Option C) is incorrect. The most common typ e in Africans is t ype A. Antimalarials such as chloroquine can precipitate an episode of haemo lysis. Heinz bodies are
oxidised, resulti ng in denatu red haemoglobin. These are removed by the spleen dur ing

ep isodes of haemolysis, causing bite and blister cells. Therefore, some of the haemolytic
process is occurring in the spleen,ie extravascular. The current picture is more compatible
with severe intravascularhaemolysis occurring very shortly after the commencement of antimalarial therapy.
Sickle cell disorder (Option D) is incorrect. Sickle tra it can protect p atients from malaria.
There are no other features on the blood film to suggest a sickle cell d isorder.
Tropical splenomegaly syndrome (Option E) is incorrect. Tropical spleno megaly syndrome is
also known as hyper-reactive malarial syndrome. This is p resent in residents of regions
where malaria is endemic. This can b e found in patients who live in endem ic areas, who have
recu rrent malarial infection and who are inadequately treated. Repeated infection leads to
an exaggerated immune response that causes massive splenomegaly,as wel l as
hepatomega ly. However, this is not associated with such a profo und anaemia, haemolysis or
acute renal impairment as observed in t his patient.


A patient has b een d iagnosed with papillary carci noma of th e thyroid. He also has a solitary
cervical lymph node. His thyroid function tests are normal.
Which one of the following is the most appropriate therapy?
A

Chemotherapy

B

Palliative care

C

Radiotherapy

D

Removal of lymph node

E

Total thyroidectomy and lymp h node dissection


Exp lanation
The answer is Tora/ rhyroidecromy and lymph node dissecrion Papillary thyroid cancer is th e most common thyroid tumour, accounting for 80% of
lesions. Distant metastases are uncommon and the overall cure rate is very high
(approaching 100%) with a combination of surgery and radio-iodi ne ablation.
A total or near-total thyroidectomy should b e performed because papillary carcinomas
are often b ilateral, and removal of thyroid t issue facilitates subsequent ab lation by radioiod ine. There is controversy regarding surgery for low-r isk unifocal papillary carcinomas
(those less than 5 cm in diameter, especially in young p atients). but bilateral resection is
associated with a lower rate of local recurrence than unilateral total lobectomy. In
p apillary ca rcinoma, th e ipsi lateral central lymph nodes should be d issected, as should
all p alpab le nodes.

Chemotherapy (Option A) is incorrect. Chemotherapy is not indicated in the routine
management of papillary thyroid cancer.
Palliat ive care (Option B) is incorrect. The prognosis with active th erapy is good.
Radiotherapy (Option C) is incorrect. External beam radiotherapy is not indicated in the
routine management of papillary thyroid cancer, although radio-iodi ne therapy is routinely
uti lised after surgical resection.
Removal of lymph node (Option D) is incorrect. Removal of the lymph node does not
address the primary tumour.

(I

A 30 -y ear-otd woman p resents to her GP w ith a 3-day history of menor rhag·a. She has no
pr evious history of m enstr ua irregu1arit y and w as p reviously well. On examination sh e is pate
and has a number of br uises on her arm s and legs. Stood tests are sent i mmediate y to t he
hospital and are reported as fo llow s:

7.5 g/ d ll (11.5-16 .5)

Hb

t5x

·o9/ t ( 4-ii x 109) w it h occasional abnormal i mmature cells reported on the

wee

blood film

Platelets

15 x 10•; 1(150-400 x 10 9 )

PT

19 s (12 -17)

APTT

45 s (24-38)

TT

22 s (14-22)

Fib rinogen

1.2 g/1 ( 2-5)

Which one of the following i s the m ost likely diagnosis?
A

Acute lymphoblastic leukaemia

B

Acute p rom yelocyr c eukaemia

C

Immune throm bocytic p .irpura

O

Meningococca septicaemia

E

Non-Hodgkin's lymphoma

Explanation

(I

The answer is Acuce promyelocytic leukaemia This lady has evidence of d isseminated intravascular coagulation ( DIC) . The clotting
times are p rolonged and the fib ri nogen is low. The next question is, what is this due to.
She also has a p ancytopen ic blood p icture, suggesting some sort of marrow
involvement. We are also told that there are occasional immature cells reported on the
b lood film. All of this suggests an underlying haematological ma lignancy for the DIC.
Acute p romyelocytic leukaemia is classical ly associated with this. It has the
chromosomal translocation t(15,17).lt is critically import ant to identify this at an early
stage to reduce the r isk of early death due to intra-cerebral haemorrhage. This is a very
treatable and curable form of leukaemia.

Acute lymphoblastic leukaemia (Option A) is incorrect. Acute lymphoblastic leukaemia can
certainly cause a p ancytopenia, bu t is not typically associated with DIC.
Immune thrombocytic purpura (Option C) is incorrect.
This would not exp lain the low haemoglobin, the low white cell count or the abnormal
clotting.
Meningococcal septicaemia (Option D) is incorrect.
Meningococcal septicaemia can certainly cause DIC. There is nothing, however, in the history
to suggest this. Such a profound pancytopenic state would not b e expected in
meningococcal septicaemia.
Non-Hodgki n's lympho ma (Option E) is incorrect.
Non-Hodgki n's lymphoma again can cause marrow invasion and can resu lt in a pancytopenic
picture if this has occurred. However, once again this is not classical ly associated with DIC,
un like acute p romyelocytic leukaemia.