Leucocytes

Question 1

Total leucocyte count reference range is:

Answer 1

4 – 11 x 109/L

Question 2

What is the reference range for neutrophils, and what proportion of WBC do they constitute?

Answer 2

2 – 6.8 x 109/L (40 – 80%)

Question 3

What is the reference range for lymphocytes, and what proportion of WBC do they constitute?

Answer 3

1 – 4 x 109/L (25 - 35%)

Question 4

What is the reference range for monocytes, and what proportion of WBC do they constitute?

Answer 4

0.2 – 1.0 x 109/L (2 – 10%)

Question 5

What is the reference range for eosinophils, and what proportion of WBC do they constitute?

Answer 5

0 – 0.5 x 109/L (0 – 5%)

Question 6

What is the reference range for basophils, and what proportion of WBC do they constitute?

Answer 6

0 – 0.2 x 109/L (0 - 2%)

Question 7

List two features of neutropaenia.

Answer 7

Highly motile cells and high risk of serious infections

Question 8

True or false: neutrophilia is rarer than neutropaenia.

Answer 8

False

Question 9

List three pathological causes of neutrophilia.

Answer 9

Acute infection, drugs, and acute haemorrhage

Question 10

The ________ ___________ of neutrophils is thought to be due to impaired cytoplasmic maturation, in the effort to rapidly generate large numbers of granulocytes.

Answer 10

Toxic granulation

Question 11

What are Döhle bodies?

Answer 11

Single or multiple blue cytoplasmic inclusions,
that are about 1-2µm long. They represent remnants of rough endoplasmic reticulum from earlier maturational stages. They are associated with myeloid ‘left shifts’, and are seen in conjunction with toxic granulation

Question 12

The term ‘_________ ________’ applies to chronic neutrophilia with marked leucocytosis (>25 x 109/L).

Answer 12

Leukaemoid reaction

Question 13

A ______________ picture indicates severe disruption of the marrow, and is common in myelofibrosis (primary or secondary).

Answer 13

Leucoerythroblastic

Question 14

_________ _________ anomaly is an autosomal dominant disorder whereby granulocyte nuclei fail to segment normally. Most granulocytes have bi-lobed nuclei, resembling bands. Cell function is normal. An acquired or ‘pseudo-Pelger-Huët’ anomaly is seen in myelodysplastic disorders and following drug therapy, and may accompany leukaemia and certain infections. Chromatin shows intense clumping, to help differentiate these cells from myelocytes,

Answer 14

Pelger-Huët

Question 15

_______________ is usually observed in allergic disorders or parasitic infection, or in asthma, eczema, drug treatment.

Answer 15

Eosinophilia

Question 16

___________ may result from viral, fungal, rickettsial, and protozoal infections.

Answer 16

Monocytosis

Question 17

______________ occurs most frequently during viral infections and only rarely in bacterial infection, except Bordatella pertusis.

Answer 17

Lymphocytosis

Question 18

Collectively, haematological malignancies account for approximately ___% of all cancers, with a considerably higher (greater than threefold) proportion of childhood cancers.

Answer 18

8

Question 19

_____________ anaemia is a mutation in the FA genes that normally helps repair DNA damage.

Answer 19

Fanconi’s

Question 20

Wiscott-Aldrich Syndrome involves mutations in the WAS gene, which affects the ability of _______ _______ to relay signals from the surface to the cytoskeleton.

Answer 20

Blood cells

Question 21

True or false: radiation is leukaemogenic.

Answer 21

True

Question 22

__________ involve ‘gain-of-function’ mutations.

Answer 22

Oncogenes

Question 23

_______-__________ are involved in a variety of important cellular processes e.g., cell-signalling pathways, directing cellular growth, differentiation and proliferation.

Answer 23

Proto-oncogenes

Question 24

Tumour-suppressor genes cause ‘loss-of-function’ _____________.

Answer 24

Mutations

Question 25

________ is a protein that interacts signalling pathways involved with cycle arrest, DNA repair, and apoptosis.

Answer 25

p53

Question 26

The term 'aneuploid' refers to:

Answer 26

Abnormal (odd) number of chromosomes, not a multiple of 23.

Question 27

__________ alterations (does not affect DNA sequence but results in a change in gene expression, e.g., methylation).

Answer 27

Epigenetic

Question 28

___________ play a role in regulating gene expression.

Answer 28

MicroRNAs

Question 29

______________ chromosome, t(9;22) BCR/ABL, is associated with CML.

Answer 29

Philadelphia

Question 30

t(15;17), retinoic acid receptor is associated with _______ _________ __________

Answer 30

Acute myeloid leukaemia

Question 31

MicroRNAs _____________ gene expression.

Answer 31

Downregulate

Question 32

_____________ stains granules of granulocytes.

Answer 32

Myeloperoxidase

Question 33

Sudan Black B stains _______ in granules.

Answer 33

Lipids

Question 34

_________ ______ ________ stains glycogen.

Answer 34

Periodic Acid Schiff

Question 35

Acid phosphatase - shows characterising staining in __-_____.

Answer 35

T-ALL

Question 36

In _______ _________ _________, the cells can be identified by using antibodies directed against markers, such as CD13, CD117, etc..

Answer 36

Acute myeloid leukaemia

Question 37

____________ __-_______ ______________ detects extra copies of genetic material.

Answer 37

Fluorescent in-situ hybridisation

Question 38

True or false: acute leukaemia often goes into remission, but may have a relapse.

Answer 38

True

Question 39

__________ leukaemia is an aggressive disease, with predominantly blast cells, some mature forms, and very few intermediate stage cells.

Answer 39

Acute

Question 40

Chronic leukaemia is less aggressive, with a predominance of more __________ cells, with <20% blasts.

Answer 40

Mature

Question 41

Acute leukaemia (AL) is defined as the presence of >20% __________ in the blood or bone marrow.

Answer 41

Blasts

Question 42

The classic triad of leukaemia is anaemia, ______________ and bleeding due to reduced levels of normal blood cells.

Answer 42

Infection

Question 43

List the three main forms of B-lymphoblastic leukaemia.

Answer 43

B-lymphoblastic leukaemia/lymphoma with translocations( t(9;22)(q34;q11.2); BCR-ABL 1) B-lymphoblastic leukaemia/lymphoma with hyperdiploidy B-lymphoblastic leukaemia/lymphoma with hypodiploidy

Question 44

List three clinical features of leukaemia.

Answer 44

Bone marrow failure, neutropaenia, and thrombocytopaenia

Question 45

True or false: normochromic-normocytic anaemia is often observed in acute leukaemia.

Answer 45

True

Question 46

List the main forms of AML.

Answer 46

AML with recurrent genetic abnormalities APL with t(15,17) (q22;q12); PML-RARA (acute promyelocytic leukaemia) AML with myelodysplasia-related changes (significant dysplasia) AML, therapy related (previous chemotherapy) AML, not otherwise specified is categorised according to morphology, cytochemistry and immunophenotype (old FAB classification) (no common genetic abnormalities) AML with minimal differentiation (no granules in cytoplasm of blasts) AML without maturation (mostly myeloblasts (Auer rods)) Acute myelomonocytic leukaemia (increase in immature neutrophils and monocytes in the bone marrow) Acute monoblastic/monocytic leukaemia (increase in immature monocytes) Acute erythroid leukaemia (immature cells of the erythroid lineage present) Acute megakaryoblastic leukaemia (abnormal megakaryoblasts)

Question 47

What are some clinical features of CML?

Answer 47

Bruising, epistaxis, and splenomegaly

Question 48

True or false: leucocytosis and increased basophils are characteristic of CML.

Answer 48

True

Question 49

CML bone marrow studies will be hypercellular, with a predominance of ___________ cells.

Answer 49

Myeloid

Question 50

CML will have a low leucocyte ___________ ____________ score.

Answer 50

Alkaline phosphatase

Question 51

CML will, in terms of molecular biology, be Ph chromosome-positive or BCR/____-positive.

Answer 51

ABL

Question 52

True or false: CML will never convert to AML.

Answer 52

False

Question 53

_____________ syndromes are serious and relatively common; they are most common in the elderly population.

Answer 53

Myelodysplastic

Question 54

List the five main types of myelodysplastic syndrome.

Answer 54

MDS with single lineage dysplasia MDS with ring sideroblasts (MDS-RS) MDS with multilineage dysplasia MDS with excess blasts MDS with isolated del(5q)

Question 55

What is the scientific term for a reduction in one or more cell lines?

Answer 55

Cytopaenia

Question 56

In some myelodysplastic syndrome varients, ring ____________ are seen in the bone marrow, due to presence of iron-loaded mitochondria.

Answer 56

Sideroblasts

Question 57

Giant, ______________ platelets are often observed in myelodysplastic syndromes.

Answer 57

Hypogranular

Question 58

In myelodysplastic syndromes, usually a macrocytic, sometimes ____________, picture is observed.

Answer 58

Dimorphic

Question 59

Anisocytosis, poikilocytosis, _____________ stippling, Howell-Jolly bodies, and NRBC may be observed in myelodysplastic syndrome.

Answer 59

Basophilic

Question 60

True or false: there is considerable overlap between leukaemias and lymphomas.

Answer 60

True

Question 61

List the two most common variants of lymphoma.

Answer 61

Hodgkin's and non-Hodgkin's

Question 62

B-cell __________ correspond to stages of maturation.

Answer 62

Neoplasms

Question 63

Pre-cursor B cells that are produced and mature in the bone marrow may undergo ___________, or develop into mature B cell.

Answer 63

Apoptosis

Question 64

B cells that have acquired a mutation give rise to B cell ________________ _____________.

Answer 64

Lymphoblastic leukaemia

Question 65

Upon encountering ___________, B lymphocytes undergo blast transformation in the lymph nodes to form effector lymphocytes.

Answer 65

Antigen

Question 66

B cells may acquire abnormality while proliferating in the __________ _____-.

Answer 66

Mantle zone

Question 67

B cells may enter the ____________ _________, where somatic hypermutations occur (GC neoplasms), and they switch from coding polyreactive antibodies into clones that express specific antibodies.

Answer 67

Germinal centres

Question 68

List two types of post-germinal centre cells.

Answer 68

Plasma cells and memory B cells

Question 69

True or false: chronic lymphocytic leukaemia is not a post-germinal centre neoplasm.

Answer 69

False

Question 70

T cell progenitors leave the bone marrow and enter the thymus where they develop into ________naïve T cells.

Answer 70

Naïve

Question 71

If an abnormality is acquired in either the bone marrow or ___________, T lymphoblastic leukaemia/lymphoma may develop.

Answer 71

Thymus

Question 72

When T cells leave the thymus and encounter antigen they undergo ________ ______________, and develop into memory and effector T cells.

Answer 72

Blast transformation

Question 73

If an abnormality is acquired in ____________ ____________ ________, there may be peripheral (mature) T cell lymphoma/leukaemia.

Answer 73

Peripheral lymphoid tissue

Question 74

List two forms of neoplasms, other than Hodgkin's lymphoma.

Answer 74

Precursor T cell neoplasm and mature T cell neoplasm

Question 75

Give three mature B cell neoplasms.

Answer 75

B cell prolymphocytic leukaemia Hairy cell leukaemia Plasma cell myeloma

Question 76

B cell chronic lymphocytic leukaemia (CLL) has a peak incidence in what age group?

Answer 76

60-80 years

Question 77

List three aetiological features of B cell chronic lymphocytic leukaemia.

Answer 77

Familial association, prolonged lifespan of B cells, and impaired apoptosis

Question 78

In B-CLL, failure of apoptosis is associated with _________________ of a known suppressor of apoptosis.

Answer 78

Upregulation

Question 79

State three clinical features of B-CLL.

Answer 79

Features of anaemia, thrombocytopaenia, and neutropaenia, lymphadenopathy, and immunosuppression

Question 80

Lymphocytosis (>5 x 109 /l, up to 300 x 109 /l), N/N anaemia (may develop due to autoimmune haemolysis), and thrombocytopaenia are laboratory findings of which neoplasm?

Answer 80

B cell chronic lymphocytic leukaemia

Question 81

List three feature that might be seen on a B-CLL blood film.

Answer 81

Smudge cells, less than 20% blasts, and inconspicuous nucleoli

Question 82

List two features of B-CLL immunophenotyping

Answer 82

B cells will have surface CD19+ Weak expression of surface IgM or IgD (monoclonal)

Question 83

_________________ replacement of normal marrow may be observed on in the bone marrow in B-CLL.

Answer 83

LymphocytiC

Question 84

True or false: translocations and trisomies, but not deletions, are seen in B-CLL.

Answer 84

False

Question 85

In B-CLL, immunoglobulin will have ____________ concentration, and will be more marked with advanced disease.

Answer 85

Reduced

Question 86

List three forms of treatment for B-CLL.

Answer 86

Immunochemotherapy (biological therapy), such as antiCD20 (Rituximab), chemotherapy, and radiation

Question 87

Malignant cells accumulate in lymph nodes, in a phenomenon known as _____________________.

Answer 87

Lymphadenopathy

Question 88

The ______________ _______ occurs when malignant cells spill over into the blood.

Answer 88

Leukaemic phase

Question 89

What is Hodgkin's lymphoma characterised by?

Answer 89

Presence of Reed-Sternberg (RS) cells

Question 90

True or false: Hodgkin's lymphoma arises from T cells.

Answer 90

False

Question 91

With what previous infection is Hodgkin's lymphoma associated?

Answer 91

Epstein-Barr virus, and particularly infectious mononucleosis

Question 92

Hodgkin's lymphoma has a ______ cure rate.

Answer 92

High

Question 93

Non-Hodgkin's lymphoma is usually derived from ___ cells.

Answer 93

B

Question 94

True or false: the aetiology of non-Hodgkin's lymphoma is poorly understood.

Answer 94

True

Question 95

______________ reflects the synthesis of Ig from a single clone of plasma cells, and is sometimes non-malignant.

Answer 95

Paraproteinaemia

Question 96

Paraproteinaemia is characterised by a _____________ immunoglobulin (Ig) band in the serum.

Answer 96

Monoclonal

Question 97

Define multiple myeloma.

Answer 97

Neoplastic proliferation of lymphocytes (B cells)

Question 98

Give three characteristics of multiple myeloma.

Answer 98

Accumulation of plasma cells in the bone marrow Presence of monoclonal protein in the serum Related organ or tissue damage (bone pain is a common presenting feature)

Question 99

Giant _____________ platelets, with functional problems, are observed is myelodysplastic syndrome.

Answer 99

Hypogranular