Chapter 1 Haemopoiesis

1.1 Which ONE of these statements is TRUE regarding normal adult bone marrow?

 A In a 50-year-old patient, the marrow consists of approximately 90% haemopoietic

cells, 10% fat

Normal ratio is 50: 50 for a patient that age.

 B It usually has a myeloid: erythroid ratio of 1: 2

Normal myeloid: erythroid ratio 2: 1 or greater.

 C Red marrow is active in the skull within adults

 D Marrow is the major source of the secretion of erythropoietin

The kidney is the dominant source of erythropoietin.

1.2 Which ONE of these statements is TRUE concerning the haemopoietic stem cell?

 A Stem cells are believed to represent approximately less than 0.001% of bone
marrow cells

It is thought to be rare – perhaps 1 in every 20 million nucleated cells in marrow.

 B The pluripotent stem cell is thought to have a CD34–CD38–CD123+ phenotype

The cells are thought to be lineage negative, CD34+, CD38–.

 C Stem cells resemble myeloblasts

They resemble a small to medium lymphocyte.

 D Humans have on average 5000–10 000 haemopoietic stem cells

Humans have 50 000–200 000 haemopoietic stem cells.

1.3 Which ONE of the following is a significant component of bone marrow stroma?

 A Monoblasts
  B Brown fat adipocytes
 C Osteoclasts
 D Epithelial cells
 E Osteoblasts

1.4 Which ONE of these is a mechanism by which granulocyte colony-stimulating factor

(G-CSF) can increase neutrophil production?

 A Increases differentiation from progenitor cells

 B Increases the self-renewal of haemopoietic stem cells

This is not a recognized mechanism.

 C Alters of lineage commitment of erythroblasts to myeloid series cells

This does not occur.

 D Enhances apoptosis of myeloid cells

Suppression occurs.

1.5 Which ONE of these cytokines has an important role in normal early haemopoiesis?

 A IL-1

Although this has an important role in initiating inflammation, it is not essential in

haemopoiesis.

 B IL-2

This stimulates T-cell division.

 C IL-3

This is an important cytokine in early haemopoiesis.

 D IL-4

This promotes generation of Th2 CD4+ T cells.

1.6 Which ONE of these is TRUE concerning the normal cell cycle?

 A DNA is replicated in G1 phase

This occurs in S phase.

 B The cell cycle is divided into the mitotic (M) phase and the interphase
 C Cyclin-dependent protein kinases (Cdk) de-phosphorylate key protein targets to
promote cell cycling

They phosphorylate.

 D Most haemopoietic stem cells cycle several times per day

These cells cycle rarely.

1.7 Which ONE of the following statements is TRUE concerning apoptosis?

 A Death receptors Fas and tumour necrosis factor receptor (TNF-R) have a death
___domain that is extracellular

The death ___domain is intracellular.

 B A critical step in apoptosis is entry of cytochrome C into mitochondria

This is released from mitochondria during apoptosis.

 C BCL-2 proteins potentiate apoptosis

They act to suppress apoptosis.

 D A final common pathway in apoptosis is action of intracellular cysteine proteases

called caspases

1.8 Which ONE of these statements is TRUE concerning transcription factors?

 A They regulate gene expression by controlling the splicing of specific genes or gene
families

They control transcription.

  B They usually contain at least two domains – the DNA-binding ___domain and the
activation ___domain
 C The product of the ABL1 gene, which is involved in the BCR-ABL1 translocation in
chronic myeloid leukaemia, is one example

ABL1 encodes a protein kinase rather than a transcription factor.

 D Helix–loop–helix is an activation ___domain

This is typically a DNA-binding ___domain.

1.9 Which ONE of these is TRUE concerning adhesion molecules?

 A Chemokines are a major family of adhesion molecule that regulates

megakaryopoiesis and platelet production

These primarily regulate migration of white cells.

 B Adhesion molecules include the BCL-2 superfamily

The BCL-2 family molecules regulate apoptosis, not adhesion.

 C Tumour cells typically do not express adhesion molecules or binding proteins

They typically do so.

 D Integrins can bind cells to extracellular matrix

1.10 Which ONE of these molecules has a role in the transduction pathway of growth
factors?

 A P53

This protein is involved in signalling the response to cellular stress and DNA damage, not
in signal transduction, and is frequently mutated in human cancer.

 B BCL-2

This molecule regulates apoptosis.

 C STAT
 This is a signal transduction molecule.

 D DNMT3A

This molecule regulates DNA methylation.

1.11 Which ONE of these statements is TRUE regarding JAK proteins?

 A Following activation, they migrate to the nucleus and bind to DNA

They lead to activation of STAT proteins.

 B The name is an abbreviation for joined activation kinase

It is an abbreviation for Janus-associated kinase.

 C Mutations in JAK2 are seen in almost all patients with polycythaemia rubra vera

This is discussed fully in the chapter on myeloproliferative neoplasms.

 C Activation of the JAK pathway generally leads to apoptosis of the cell

JAK signalling generally leads to cellular survival and proliferation.

1.12 Which ONE of these statements is most accurate regarding the major sites of
haemopoiesis during development?

 A The spleen is an active site of haemopoiesis in infancy

The spleen is a site of haemopoiesis in the fetus but not after birth.

 B The liver and bone marrow are equally active during gestation

The liver is more important in gestation and the bone marrow becomes the predominant
source of haemopoiesis 2 months after birth.

 C Haemopoiesis starts in the liver and yolk sac and moves to the bone marrow only
after birth

The liver is seeded after a few months’ gestation. The yolk sac begins during early
embryogenesis. Bone marrow haemopoiesis starts prior to birth.
  D It starts in the yolk sac, moves to the liver and then to the bone marrow

The yolk sac is the most important in the first few weeks of embryonic life.

Chapter 2 Erythropoiesis and general aspects

of anaemia
2.1 Which ONE of these statements is TRUE about haemoglobin?

 A One molecule of haemoglobin contains one atom of iron

It contains four atoms.

 B Haemoglobin is broken down in macrophages

 C Haemoglobin is different in children and adults

Both are dominantly HbA.

 D Normal adult blood contains two types of haemoglobin

There are three types: HbA, HbF and HbA2.

2.2 Which ONE of these statements is TRUE about reticulocytes?

 A Reticulocytes contain more DNA than RNA

They contain RNA not DNA; the nucleus has been expelled.

 B The proportion of circulating reticulocytes is raised in megaloblastic anaemia

It is usually decreased.

 C They are permanently raised after splenectomy

 D They are raised after haemorrhage

The marrow's response to anaemia.

2.3 Which ONE of these statements is TRUE concerning erythropoietin?

 A 90% of erythropoietin is made in the liver

90% is made in the kidney. Thrombopoietin is made in the liver.

 B Germline mutation of a hypoxia response gene may result in lower plasma levels
of erythropoietin and anaemia

Typically plasma levels are higher and polycythaemia results.

 C One stimulus to production is a low atmospheric oxygen level

 D Levels in blood are high if a tumour secreting erythropoietin is causing
polycythaemia and also in severe renal disease

Levels are low in severe renal disease.

2.4 Which ONE of these statements is TRUE concerning the therapeutic use of
erythropoietin?

 A Adverse effects include hypotension and bleeding disorders

Hypertension and thrombosis are concerns.

 B The main indications are in patients with aplastic anaemia and leukaemia

Most use is in chronic renal failure. Use is typically ineffective.

 C Oral or parenteral iron is often needed to optimize the response

 D Recombinant erythropoietin may be administered orally on a daily basis

It is given subcutaneously or intravenously, less frequently.

2.5 Which ONE of these statements is TRUE concerning the biochemistry of haemoglobin?

 A Haemoglobin is ∼85% saturated in arterial blood

It is usually > 95% in arterial blood, and 70% in venous blood.

 B As the haemoglobin molecule loads and unloads O2 in hypoxic tissues, the

individual globin chains in the haemoglobin molecule move with respect to each other
 This is true and leads to the classic sigmoid dissociation curve.

 C When O2 is unloaded the β chains are pulled apart, permitting entry of the
metabolite 2,3‐diphosphoglycerate (2,3‐DPG) and resulting in a higher affinity of the
molecule for O2

The affinity is lower.

 D The P50 (i.e. the partial pressure of O2 at which haemoglobin is half saturated with
O2) of normal blood is 40 mmHg

The P50 is 26.6 mmHg.

2.6 Which ONE of these conditions is described by this phrase? ‘A clinical state in which
circulating haemoglobin is present with iron in the oxidized (Fe3+) instead of the usual Fe2+
state.’

 A Unstable haemoglobin

Not a form of intravascular haemolysis.

 B Methaemoglobinaemia
 C Blackwater fever

Not a form of intravascular haemolysis.

 D Glucose‐6‐phosphate dehydrogenase deficiency

Not a form of intravascular haemolysis.

2.7 Which ONE of these statements is TRUE concerning the red cell?

 A Excess membrane leads to a spheroid instead of discoid shape

This is a result of loss of membrane.

 B It has an average diameter of 3.5 µm and can only pass through vessels of this size
or larger

It has a diameter of 8 µm, but is very flexible so that it can pass through capillaries of
3.5 µm.
  C It generates energy as adenosine triphosphate (ATP) by the anaerobic glycolytic
(Embden–Meyerhof) pathway

This is true.

 D It generates oxidizing power as nicotinamide adenine dinucleotide phosphate

(NADPH) by the Embden–Meyerhof pathway

This generates reducing power; NADPH is generated by the monophosphate shunt.

2.8 Which ONE of these statements is TRUE concerning the red cell membrane?

 A More than 50% of the membrane is cholesterol

50% is protein, 20% cholesterol, 20% phospholipids and 10% carbohydrates.

 B Spectrin and ankyrin are extracellular proteins that reinforce the membrane

They are intracellular.

 C Alterations in serum lipids do not influence the red cell membrane

These can occur (e.g. in advanced liver disease).

 D Inherited defects in membrane proteins can cause hereditary spherocytosis and

elliptocytosis

Chapter 3 Hypochromic anaemias

3.1 Which ONE of the following normally contains > 10% of body iron?

 A Transferrin

This iron transport protein only accounts for a tiny amount of total body iron stores
(<5 mg).

 B Myocardial cells
 Most iron is stored in macrophages and liver parenchyma. The heart stores little iron
except in haemochromatosis.

 C Intracellular cytochromes

They contain iron but much less body iron.

 D Macrophages

A sizeable iron pool is stored as ferritin and haemosiderin.

3.2 Which ONE of the following statements is TRUE about sideroblastic anaemia?

 A Inherited forms are more common in females

Inherited forms are often X‐linked and therefore are more common in males.

 B Ring sideroblasts in myelodysplastic syndromes are associated with genetic

alterations in TP53

Almost all patients with MDS and sideroblastic anaemia have SF3B1 spliceosome
mutations.

 C It may be caused by folate deficiency

There is no association with folate deficiency.

 D It may respond to pyridoxine

It may respond in the inherited forms.

3.3 Which ONE of the following statements about iron distribution is TRUE?

 A A unit of packed red blood cells typically contains 200–250 mg iron

This is true.

 B A man needs to absorb about 10 mg of dietary iron daily

Average absorption is 1–2 mg/day.

 C A molecule of transferrin may transport up to four atoms of iron

 Two atoms maximal.

 D Poor diet is the major cause of iron deficiency in the UK

Haemorrhage is the main cause.

3.4 Which ONE of the following may be used to treat iron deficiency?

 A Ferric sulfate

Ferrous sulfate is needed.

 B Desferrioxamine

This removes (chelates) iron.

 C Ferrous gluconate
 D Vitamin C monotherapy

This enhances iron absorption, but does not alone correct the deficiency.

3.5 Which ONE of the following statements regarding oral iron therapy is TRUE?

 A Slow‐release oral iron preparations are indicated for patients with gastrointestinal
disorders

Slow‐release preparations are never clinically indicated.

 B Treatment with oral iron should be given together with vitamin C

This is commonly done, but is not a standard or essential practice.

 C Iron supplementation orally commonly causes bloating and constipation

Gastrointestinal adverse effects are the most common.

 D Total body iron stores can usually be replaced with oral iron in 3–4 weeks

Longer therapy is usual, up to 3–6 months.

 E Iron supplementation is routinely indicated in pregnancy

 Many women need supplementation, but not all.

3.6 Which ONE of the following is a feature of iron deficiency?

 A Raised serum hepcidin

Hepcidin is reduced.

 B Raised serum transferrin

 C Raised mean corpuscular volume (MCV)

MCV is typically normal or reduced.

 D Raised serum ferritin

Ferritin is usually low or in the presence of inflammation may be normal.

3.7 In which ONE of the following conditions is iron absorption favoured?

 A Pregnancy
 B Chronic inflammation

Inflammation decreases iron absorption.

 C Transfusional iron overload

Genetic haemochromatosis is associated with increased iron absorption in most cases, but
in transfusional iron overload absorption is not increased.

 D Ageing

3.8 Which ONE of the following statements is TRUE about serum iron levels?

 A It is typically low in inflammatory diseases

 B When serum iron levels are high, hepcidin levels are typically low

This is variable, affected e.g. by inflammation as well as iron status. Typically when iron
levels are high so are hepcidin levels.

 C Levels of serum iron tend to be higher in the evening

 Usually higher in the morning.

 D Serum iron is transported in plasma by ferritin

It is transported by transferrin.

Chapter 4 Iron overload

4.1 Which ONE of these statements is TRUE about genetic haemochromatosis?

 A It is usually dominantly inherited

The most common forms are autosomal recessive.

 B It is most commonly caused by mutation of the HFE gene

 C It causes excess iron deposition in the duodenal mucosa

Iron is absorbed by the duodenum, but stored elsewhere.

 D Macrophage iron is typically markedly increased

Most of the time due to low hepcidin macrophage ferroportin expression is increased and
these cells have relatively low iron concentrations.

4.2 Which ONE of the following organs is least commonly damaged by transfusional iron
overload?

 A Endocrine cells of the pancreas

These can be damaged.

 B Kidneys

The kidneys are not typically damaged in iron overload.

 C Parathyroid glands

These can be damaged.

  D Pituitary gland

This can be damaged.

 E Heart

This can be damaged.

4.3 For which ONE of the following conditions is iron chelation therapy LEAST frequently
indicated?

 A Sickle cell disease

These patients may develop iron overload from repeated transfusions.

 B Genetic haemochromatosis

Most patients are not anaemic and can safely undergo phlebotomy.

 C Thalassaemia major

Due to chronic anaemia and increased iron absorption, for these patients chelation
therapy is essential.

 D Thalassaemia intermedia

Due to chronic anaemia and increased iron absorption, for many of these patients
chelation therapy is required.

4.4 Which ONE of the following is most useful for monitoring transfusional iron overload?

 A Bone marrow aspirate

Not useful. Absent marrow iron staining is a marker of iron deficiency, but this is not a
useful assay for iron overload.

 B Serum transferrin

Ferritin is useful, but transferrin itself does not track total body iron stores.

 C Magnetic resonance imaging

 Currently the most useful technique for measuring cardiac and liver iron burden.

 D Lung function

Lung function testing is not helpful for monitoring iron overload.

4.5 Mutations in which of the following genes is associated with autosomal recessive
juvenile haemochromatosis?

 A HJV (hemojuvelin)
 B HFE

This is the most common cause of classical hereditary haemochromatosis diagnosed in

adulthood.

 C SLC40A1 (ferroportin)

This is most commonly associated with autosomal dominant genetic haemochromatosis

with marked increase in macrophage iron.

 D SF3B1 (splicing factor)

Acquired mutations in this gene are associated with myelodysplastic syndromes with ring
sideroblasts, which can result in iron overload with repeated transfusions.

4.6 Which of the following is a side‐effect of desferrioxamine iron chelation therapy?

 A Agranulocytosis

A side‐effect of deferiprone.

 B Deafness

Especially in children and with high doses of the drug.

 C Abnormal liver function

Not a side‐effect.

 D Abnormal renal function

Not a side‐effect
Chapter 5 Megaloblastic anaemias and other
macrocytic anaemias
5.1 Which ONE of the following supplements is used therapeutically?

 A Adenosylcobalamin

This is an unstable form that is not used clinically.

 B Polyglutamate folate

This is a natural form of folate in the diet, but the fully oxidized monoglutamate form of
folate, folic acid, is used in dietary supplements.

 C Hydroxocobalamin

Commonly used to treat vitamin B12 deficiency. This is also the type of vitamin B12 found
in food.

 D Methyltetrahydrofolate

This is the form of folate used at the cellular level for regulation of homocysteine and for
DNA reproduction.

5.2 Which ONE of the following is associated with pernicious anaemia?

 A Ileal resection

Although this can be a cause of vitamin B12 deficiency due to malabsorption, pernicious
anaemia is due to deficiency of a different aetiology.

 B Elevated serum gastrin

 C Pregnancy

Vitamin B12 needs are higher during pregnancy, but pernicious anaemia is a specific
diagnosis.

 D Malabsorption of B12–intrinsic factor complex

 The problem in pernicious anaemia is with production of intrinsic factor due to anti‐
parietal cell or anti‐intrinsic factor antibodies, not malabsorption.

5.3 Which ONE of these statements is TRUE concerning the reduction of folate?

 A The biochemical reaction requires vitamin B12

 B Reduction is inhibited by sulphonamide
 C Reduction is inhibited by methotrexate
 D Reduction occurs during thymidylate synthesis

5.4 Which ONE of the following drugs causes folate deficiency?

 A Valproic acid

The mechanism is not clear.

 B Metformin

This causes B12 deficiency.

 C Prednisolone

This is not a known cause of folate deficiency.

 D Zinc gluconate

Excessive use can cause copper deficiency.

5.5 Causes of microcytic red cells include which ONE of the following?

 A Alcohol

Red cells are usually larger (macrocytic).

 B Renal disease

Typically normocytic red cells.

 C α‐Thalassaemia
 D Increased reticulocyte count
 Reticulocytes are larger than mature red cells and so the mean corpuscular volume
(MCV) is increased.

5.6 Which ONE of the following statements is TRUE about megaloblastic anaemia?

 A It is always caused by vitamin B12 or folate deficiency

There are rare inborn metabolic errors that cause it.

 B Bone marrow appearances are distinct in vitamin B12 or folate deficiency

They are identical.

 C It is caused by defective DNA synthesis

 D It may be caused by nitric oxide

Nitrous not nitric oxide inhibits B12 metabolism and causes megaloblastic anaemia.

5.7 Which ONE of the following is caused by folate deficiency?

 A Spinal cord posterior column degeneration

This is a consequence of severe vitamin B12 deficiency.

 B Haemolytic anaemia
 C Neural tube defect

It is associated with folate deficiency in the mother.

 D Duodenal atrophy

5.8 Which ONE of the following can contribute to vitamin B12 deficiency?

 A Haemolytic anaemia

This may lead to folate deficiency.

 B Veganism

If all animal products are avoided.

  C Metastatic carcinoma
 D Jejunal resection

Because B12 is absorbed through the ileum.

5.9 Which ONE of the following clinical findings is caused by vitamin B12 deficiency?

 A Vitiligo

Although vitiligo is sometimes seen in association with pernicious anaemia, it is a result

of a separate autoimmune process.

 B Cerebellar ataxia
 C Dementia
 D Abnormal liver function

Chapter 6 Haemolytic anaemias

6.1 Spherocytes in the blood film are a feature of which ONE of the following conditions?

 A Thalassaemia major

Target cells are more typical.

 B Autoimmune haemolytic anaemia

 C Reticulocytosis

Polychromasia is characteristic.

 D Glucose‐6‐phosphate dehydrogenase (G6PD) deficiency

Heinz bodies are commonly associated with G6PD deficiency, with ‘bite’ cells noted
during acute haemolytic episodes.

6.2 Which ONE of the following is a typical feature of a chronic extravascular haemolytic
anaemia?
  A Raised serum conjugated bilirubin

Bilirubin from haemolysis is mostly unconjugated.

 B Low reticulocyte count

In the presence of a healthy marrow that can respond adequately, reticulocytes are
typically raised because of the chronic anaemia.

 C Hypocellular bone marrow

The cellularity is usually increased, compensating for the anaemia.

 D Risk for gallstones

Pigment gallstones are commonly observed with chronic haemolysis.

6.3 Which ONE of the following is a cause of intravascular haemolysis?

 A Thalassaemia major

This typically causes extravascular haemolysis, mostly in the spleen.

 B Transfusion of an ABO‐incompatible red cell unit

 C Autoimmune haemolytic anaemia

This is usually extravascular and antibody mediated.

 D Rh incompatibility between donor and recipient

This causes extravascular (cells are destroyed in the spleen) haemolysis.

6.4 Which ONE of the following is a typical feature of intravascular haemolysis?

 A Positive Coombs test

Typically seen in autoimmune causes (extravascular).

 B Haemosiderin in urine
 C Elevated haptoglobin level
 Haptoglobin can be reduced in all forms of haemolysis, but especially intravascular.

 D Raised serum bilirubin

This is usually normal.

6.5 Which ONE of these statements is TRUE regarding hereditary spherocytosis?

 A It is caused by an inherited defect in haemoglobin

It is a membrane defect.

 B It is more common in males

It is autosomally inherited in most cases and approximately equal between the sexes.

 C It can be treated by splenectomy

Splenectomy can be beneficial, although it is not needed in all cases.

 D It is more frequently encountered in southern than northern Europe

The incidence is higher in northern Europe.

6.6 Which ONE of the following statements is TRUE about autoimmune haemolytic
anaemia?

 A It may be due to beta‐blocker drugs

These are not drugs that cause it.

 B It is associated with pernicious anaemia

Pernicious anaemia is associated with autoimmunity, but usually for thyroid and other
organs, not haemolysis.

 C It is frequently associated with immunoglobin (Ig) A autoantibodies in serum

Usually it is driven by IgG or IgM autoantibodies.

 D It may complicate B‐cell chronic lymphocytic leukaemia

  E It commonly complicates malaria

Parasitic infections usually cause intravascular haemolysis that is non‐immune mediated.

6.7 Which ONE of the following statements is TRUE about glucose‐6‐phosphate

dehydrogenase (G6PD) deficiency?

 A It usually presents as a chronic haemolytic anaemia

This can happen but is a rare presentation.

 B Certain foods are the most common precipitant of haemolysis worldwide

Infection is the most common precipitant, followed by drugs.

 C Deficiency provides some protection against types of Clostridium infection

It protects against malaria, especially Falciparum, not other organisms.

 D Carrier females have approximately 50% of normal G6PD levels

 E Sulphonamides, e.g. Septrin, are safe to administer in patients with this deficiency

These drugs can cause haemolysis and are contra‐indicated.

Chapter 7 Genetic disorders of haemoglobin

7.1 Which ONE of the following is a characteristic feature of thalassaemia intermedia?

 A It may be due to homozygous βo thalassaemia

This is a cause of thalassaemia major.

 B Crystallization of haemoglobin within red cells is characteristic

 This is more typical of haemoglobinopathies like sickle cell disease or haemoglobin SC.

 C It may not be associated with a normal haemoglobin level

By definition anaemia is present.

 D It is associated with bone deformities

Due to marrow expansion.

7.2 Which ONE of these statements is TRUE about β‐thalassaemia major?

 A The major cause of death in the contemporary era is liver failure

The major cause of death without iron chelation is cardiac iron overload. In those who
receive adequate chelation, it is infection.

 B A diagnosis of thalassaemia major means iron chelation should begin immediately

at diagnosis

This is only necessary after a number of blood transfusions, usually 10–20, have been
given.

 C It is usually caused by deletion of β globin genes

Unlike α‐thalassaemia, β‐thalassaemia is usually caused by point mutations in globin

genes, not gene deletions.

 D Antenatal diagnosis and prevention are reducing the incidence

Genetic counselling of high‐risk populations and prenatal diagnosis by DNA analysis on

cord blood or chorion villous biopsy are reducing new cases and have virtually
eliminated them in some countries such as Cyprus.

7.3 Which ONE of these statements is TRUE about β‐thalassaemia major?

 A It presents at birth

It typically presents around month 3–6 or later when the switch from γ‐ to β‐globin gene
usage occurs.

 B It is aggravated by high fetal haemoglobin production

 This ameliorates the disease.

 C It is most common in tropical and semi‐tropical regions of the world

 D It is associated with an increased risk of bone infarction

Osteoporosis and bone expansion are often present, but infarction is more characteristic
of sickle cell disease.

7.4 Which of the following statements is TRUE about α‐thalassaemia?

 A Haemoglobin H disease is a form of α‐thalassaemia

This is deletion of three of four α genes.

 B α‐Thalassaemia causes a macrocytic, hypochromic blood picture

This is false – microcytic, hypochromic.

 C Concomitant inheritance of α‐thalassaemia makes β‐thalassaemia more severe

It improves this because there is a better α: β chain balance.

 D It typically does not present until after month 6 of life

It can cause death in utero. Hydrops fetalis is a result of deletion of all four α genes.

 E It is most frequent in the Middle East

It also occurs in other tropical areas and most commonly in the Far East.

7.5 Which ONE of the following statements is TRUE about β‐thalassaemia trait?

 A It is associated with a raised haemoglobin A2 level

 B It is associated with iron overload

This does not occur, only in thalassaemia intermedia and major.

 C It is associated with a reticulocytosis

This also does not occur.

  D It is associated with splenomegaly

This does not occur in trait, only in thalassaemia intermedia and major.

7.6 Which ONE of these statements is TRUE concerning sickle cell trait?

 A Sickle cell trait may cause mild anaemia

It is largely asymptomatic, with normal haemoglobin levels.

 B Sickle cell trait protects against severe malaria in endemic regions

 C Sickle cell trait occurs mainly in females

Incidence is equal between sexes.

 D Sickle cells can usually be observed in the peripheral blood

Usually there is no morphological change in trait, only in disease.

7.7 Which ONE of the following statements is TRUE about sickle cell anaemia?

 A The oxygen dissociation curve is shifted to the left (i.e. the haemoglobin gives up
oxygen less easily than normal)

It is actually shifted to the right.

 B It is associated with stunted growth

Unlike thalassaemia major, this is not a major feature.

 C It may cause ankle ulcers despite preserved flow in large arteries to the distal legs
 D The occurrence of stroke associated with sickle cell disease is not predictable

Serial monitoring of intracranial blood flow can predict and exchange transfusion in high‐
risk children can prevent stroke.

 E When homozygous or co‐inherited with haemoglobin C, haemoglobin S is

associated with atrophy of the spleen in the first 5 years of life

This is true of Hb SS, but the spleen is usually preserved until much later in Hb SC.
7.8 Which ONE of the following occurs frequently in children with sickle cell disease (Hb
SS)?

 A Splenomegaly

Splenic infarcts and atrophy usually occur early in the disease.

 B Extramedullary haemopoiesis

This is not typical – it is more common in thalassaemia major.

 C Silent cerebral infarction

This is common and may be subclinical.

 D Small bowel infarction due to mesenteric ischaemia

This is rare.

Chapter 8 The white cells, part 1:

granulocytes, monocytes and their benign
disorders
8.1 Which ONE of the following is TRUE about neutropenia?

 A Patients with severe neutropenia must always be kept isolated in a high‐efficiency

particulate air‐filtered room

Most infections in neutropenic patients occur from endogenous flora in mouth, bowel etc.
and filtered rooms help little. Hospitals may choose to use such rooms, but patients do
not need to be isolated at home.

 B Neutropenia commonly occurs in aplastic anaemia

This is a common disease feature.

 C It may be caused by aspirin

 Aspirin is not a typical cause of neutropenia or other cytopenias.

 D It is associated with myeloproliferative neoplasms

Myeloproliferative neoplasms often cause neutrophilia.

8.2 A 45‐year‐old woman presents with neutropenia, positive rheumatoid factor, preserved
platelet count and splenomegaly. What is the most likely diagnosis?

 A Systemic lupus erythematosus

Positive rheumatoid factor (RF) and splenomegaly are not characteristic of SLE.

 B Acute myeloid leukaemia

Normal platelet count is rare in AML, and RF is not characteristic.

 C Felty syndrome
 D Gaucher disease

This may present with splenomegaly, but the other features mentioned are less frequent.

8.3 Which ONE of the following is a cause of neutrophil leucocytosis?

 A Hypothyroidism

This rarely causes macrocytic anaemia. Drugs used for hyperthyroidism can cause
neutropenia.

 B Influenza

This viral infection causes neutropenia and lymphocytosis.

 C Asthma

Typically eosinophilia is seen, not neutrophilia.

 D Corticosteroid therapy

Common feature.

8.4 Which ONE of the following is a common cause of eosinophilia?

  A Primary myelofibrosis

This causes a neutrophil or basophil increase.

 B Eczema

Eosinophilia is typical with allergic diseases.

 C Streptococcal infection

Parasitic infections often cause eosinophilia, but not other types of infection.

 D Acute myeloid leukaemia

Myeloproliferative neoplasms and some cases of chronic myeloid leukaemia are

associated with eosinophilia, but blood eosinophilia is rare in AML.

8.5 Which ONE of the following is a characteristic cause of a leuco‐erythroblastic blood

film?

 A Primary myelofibrosis

Such a pattern is typical.

 B Aplastic anaemia

Usually the blood film shows pancytopenia in this condition.

 C Miliary tuberculosis

This can cause cytopenias, but the leuco‐erythroblastic pattern is rare.

 D Polycythaemia vera

This only causes a leuco‐erythroblastic picture when fibrosis evolves and it becomes
post‐polycythaemic myelofibrosis.

8.6 Which ONE of the following may be seen in the cytoplasm of neutrophils?

 A Malarial parasites

These are typically found within red cells.

  B Döhle bodies

These are often found in neutrophil cytoplasm in the presence of severe infection.

 C Siderotic granules

These are typically found in red cells.

 D Pelger–Hüet abnormality

This is a nuclear anomaly, not cytoplasmic.

8.7 Manifestations of Gaucher disease may include which ONE of the following?

 A Spontaneous fractures

Bone lytic lesions (‘Ehrlenmeyer flask’) can be seen, but fractures are rare.

 B Visual impairment

This is not characteristic.

 C Hypergammaglobulinaemia
 D Monocytosis

Patients usually do not have an elevated monocyte count; they may have neutropenia.

PREPARED BY LORD ZUCKERMAN 2020.