Lymphoid Neoplasms

Question 1

Is lymphoma a common malignancy?

Answer 1

Yes - 6th most commonly diagnosed cancer

Question 2

How should lymph nodes be sent to the pathology lab for testing?

Answer 2

Fresh, with no formalin or other fluids in tub

Question 3

Where are T cells made?

Answer 3

Bone marrow

Question 4

Where do T cells mature?

Answer 4

Thymus

Question 5

Where do T cells become activated?

Answer 5

Secondary lymphoid organs

Question 6

Where are B cells made?

Answer 6

Bone marrow

Question 7

Where do B cells mature?

Answer 7

Bone marrow

Lymphoid organs

Question 8

Where do B cells become activated?

Answer 8

Lymphoid follicles

Paracortex

Question 9

Where does the lymph enter a lymph node?

Answer 9

Afferent lymphatic vessel > marginal sinus

Question 10

Where are the follicles and germinal centres located in the lymph node?

Answer 10

In cortex

Question 11

Where do T cell responses occur in the lymph node?

Answer 11

Paracortex

Medulla

Question 12

What is contained within the medulla of the lymph node?

Answer 12

T cells

Medullary sinuses

Question 13

What is the translocation in Burkitt lymphoma?

Answer 13

t(8;14)

Question 14

How is the t(8;14) translocation involved in the pathogenesis of Burkitt lymphoma?

Answer 14

c-MYC gene on chromosome 8 translocated to Ig gene on chromosome 14

Question 15

What is the translocation in follicular lymphoma?

Answer 15

t(14;18)

Question 16

How is the t(14;18) translocation involved in the pathogenesis of follicular lymphoma?

Answer 16

Heavy chain locus on chromosome 14 translocated to Bcl-2 locus on chromosome 18 > increased expression of Bcl-2 > increased anti-apoptotic activity

Question 17

Wat are the characteristic cells seen in Hodgkin’s lymphoma?

Answer 17

Reed-Sternberg cells

Question 18

What methods can be used to identify the CD antigens on cells?

Answer 18

Immunohistochemistry
Flow cytometry
In situ hybridisation

Question 19

What investigations can be used to confirm that a monoclonal population of cells is present?

Answer 19

All molecular techniques based on clonal proliferation

• PCR
• FISH
• Immunohistochemistry
• Flow cytometry

Question 20

What does immunohistochemistry identify?

Answer 20

Panel of Ag markers to detect surface markers and other proteins
Detect EBV DNA
Can correlate with histology

Question 21

What does flow cytometry identify?

Answer 21

Analysis of fluorochrome labelled cells
Detects surface markers for immunophenotyping
Detects B cell clonality
No correlation with histology

Question 22

What does PCR identify?

Answer 22

Assesses monoclonality by assessing IgH gene/T cell receptor gene rearrangements
Identify specific translocations

Question 23

What does FISH identify?

Answer 23

Specific translocations and other genetic abnormalities

Question 24

A blood smear showed increased numbers of small mature lymphocytes. In the context of haematological malignancies, what is the likely diagnosis?

Answer 24

Chronic lymphocytic leukaemia

Question 25

What test would be needed to confirm the diagnosis of chronic lymphocytic leukaemia?

Answer 25

FBE Liver/spleen ultrasound Bone marrow biopsy

Question 26

What is the likely cause of lymphadenopathy and splenomegaly in the context of haematological malignancies?

Answer 26

Malignant infiltrate

Question 27

What is CD19 a marker of?

Answer 27

B cells

Question 28

What is CD10 a marker of?

Answer 28

Follicular lymphoma

Question 29

What are the management options for someone with chronic lymphocytic leukaemia?

Answer 29

Monitor Treatment - Chemotherapy - Immunotherapy

Question 30

What is rituximab?

Answer 30

mAb against CD20

Question 31

What is ZAP-70 and what is its relevance in chronic lymphocytic leukaemia?

Answer 31

If present, poor prognosis in CLL

Question 32

What is Richter's transformation?

Answer 32

Transformation of small cell lymphomas/CLL into diffuse large B cell lymphoma

Question 33

What are the symptoms of superior vena cava syndrome?

Answer 33

``` Headache Facial oedema Facial congestion Shortness of breath Distended veins in upper limbs and neck ```

Question 34

What causes superior vena cava syndrome?

Answer 34

Compression of superior vena cava

Question 35

How much of the lymph node can be neoplastic in Hodgkin's lymphoma?

Answer 35

Up to 5%

Question 36

What is the pathogenesis of Hodgkin's lymphoma?

Answer 36

Clonal proliferation of B cells of germinal centre origin Produce cytokines > attract other inflammatory cells EBV present in 40-80% Other genetic risk factors

Question 37

What are some examples of EBV related diseases?

Answer 37

Infectious mononucleosis Lymphoproliferative disorders Nasopharyngeal carcinoma

Question 38

What are some lymphoproliferative disorders related to EBV?

Answer 38

``` Burkitt lymphoma Plasmablastic lymphoma DLBCL Haemophagocytic lymphohistiocytosis Post-transplant lymphoproliferative disorder ```

Question 39

What are the risk factors for lymphoma?

Answer 39

``` Many arise de novo Immunosuppression/immunodeficiency Some autoimmune diseases EBV H pylori Other infectious agents Chemotherapy Radiation Chemical exposure ```

Question 40

What is the usual site of non-Hodgkin's lymphomas?

Answer 40

Usually arise in lymphoid organs Many extranodal; eg: - Thyroid in Hashimoto's disease - Stomach in H pylori gastritis

Question 41

What is the usual site of Hodgkin's lymphomas?

Answer 41

Initially enlargement of 1 lymph node group | May progress to involve adjacent lymph node groups and other lymphoid organs

Question 42

What are the systemic symptoms of lymphoma?

Answer 42

Fever Weight loss Night sweats

Question 43

A 65-year-old man presents with a several month history of generalised lymphadenopathy. A lymph node biopsy demonstrates effacement of normal architecture which is replaced by nodular proliferations of predominantly small cleaved lymphocytes resembling germinal centre B cells. A (14;18) translocation in the malignant cells is identified. What is the lymphoma?

Answer 43

Burkitt lymphoma

Question 44

A 24-year-old female presented with a 6 week history of fever, weight loss, shortness of breath, and cervical lymphadenopathy. Two months earlier she had spent 2 weeks in Vietnam. Chest x-ray revealed an anterior mediastinal mass. Reed-Sternberg cells are seen in the biopsy. What is the diagnosis?

Answer 44

Hodgkin lymphoma