Lymphoid malignancies

Question 1

How can lymphomas present?

Answer 1

Enlarged lymph nodes (Lymphadenopathy)
Extranodal involvement
Bone marrow involvement
Systemic (B) symptoms - Weight loss >10% in 6 months, fever, night sweats, pruritis, fatigue

Question 2

How is lymphoma diagnosed?

Answer 2

Biopsy to tell us the type

Clinical examination and imaging for staging

Question 3

Malignancy in which cells causes acute lymphoblastic leukaemia?

Answer 3

Lymphoid progenitor/immature lymhpoid cells

Question 4

Malignancy in which cells cause chronic lymhpoid leukaemia and lymphomas?

Answer 4

Mature lymphoid cells - B cells and T cells

Question 5

What is non-hodgkin lymphoma?

Answer 5

Any lymphoma that isn’t Hodgkin lymphoma

Question 6

What is ALL?

Answer 6

Cancerous disorder of lymphoid progenitor cells

Question 7

What is the normal pathway of lymphoid progenitors?

Answer 7

Immature, rapidly proliferating cells differentiating into lymphocytes

Question 8

What happens in leukaemia?

Answer 8

No differentiation, instead, rapid, uncontrolled growth and accumulation

Question 9

Where does ALL usually present?

Answer 9

Usually in bone marrow but can be anywhere

Question 10

How does ALL present?

Answer 10

2-3 week history of bone marrow failure or bone/joint pain

Weight loss

Question 11

What is the most common form of ALL?

Answer 11

B-cell lineage

Question 12

What blood counts would you expect from ALL?

Answer 12

Very low haemoglobin
Extraordinarily high white cell count
Low platelets

Question 13

What are characteristics of malignant cells in ALL?

Answer 13

Large cells
Express CD19 marker -all B-cells have this
Express CD34 and TDT - markers of very early, immature cells

Question 14

What is standard treatment for ALL?

Answer 14

Induction chemotherapy to obtain remission
Consolidation therapy
CNS directed treatment
Maintenance treatment for 18 months
Stem cell transplantation if high risk - adult patient
Bi-specific T-cell engagers (BiTe molecules) - blinatumumab
Chimeric antigen receptor T-cells

Question 15

What is Chimeric antigen receptor T-cells treatment?

Answer 15

Patient/healthy 3rd party T-cells harvested
Transfected to express a specific T-cell receptor expressed on leukaemia cells (CD19)
Expanded in vitro
Re-infused into patient

Question 16

What are key side effects of CAR T-cell treatment?

Answer 16

Cytokine release syndrome - fever, hypotension, dyspnoea

Neurotoxicity

Question 17

What are ALL poor risk factors?

Answer 17

Increasing age
Increased white cells
Cytogenetics/molecular genetics
Slow/poor response to treatment

Question 18

What is Chronic lympoid leukaemia?

Answer 18

Mature lymphoid cells, grows slowly, requires a lymphocyte count >5

Question 19

How does CLL typically present?

Answer 19

Often assymptomatic at presentation
Bone marrow failure - anaemic/thrombocytopenic
Lymphadenopathy
Splenomegaly
Fever and sweats
Immune paresis
Haemolytic anaemia

Question 20

What is immune paresis?

Answer 20

Loss of normal immunoglobulin production

Question 21

What staging system is used for CLL?

Answer 21

Binet system

Question 22

What is stage A CLL?

Answer 22

<3 lymph node areas

Question 23

What is stage B CLL?

Answer 23

3 or more lymph node areas

Question 24

What is stage C CLL?

Answer 24

Stage B + anaemia or thrombocytopenia

Question 25

What are indications for treatment of CLL?

Answer 25

Progressive bone marrow failure
Massive lymphadenopathy
Progressive splenomegaly
Lymphocyte doubling time <6 months or >50% increase over 2 months
Systemic symptoms
Autoimmune cytopenias

Question 26

How is CLL treated?

Answer 26

Often nothing - watch and wait
Cytotoxic chemotherapy
Monoclonal antibodies
Novel agents

Question 27

What are examples of cytotoxic chemotherapy drugs used in CLL?

Answer 27

Fludarabine

Bendamustine

Question 28

What are examples of monoclonal antibodies used in CLL?

Answer 28

Rituximab

Obinatuzumab

Question 29

What are poor prognostic markers for CLL?

Answer 29

Advanced disease (Binet B or C)
Atypical lymphocyte morphology
Rapid lymphocyte doubling time <12 months
CD38+ expression
Loss/mutation of P53
Unmutated IgVH gene

Question 30

What are the 4 stages of lymphoma?

Answer 30

Stage I - One site
Stage II - One other site
Stage III - Many sites only lymph nodes
Stage IV - Extranodal involvement

Question 31

What staging system is used for lymphoma?

Answer 31

Ann Arbor

Question 32

How is non-Hodgkin lymphoma classified?

Answer 32

Lineage - B or T cell

Grade

Question 33

What is low grade lymphoma?

Answer 33

Indolent, often asymptomatic

Responds to chemotherapy but incurable

Question 34

What is high grade lymphoma?

Answer 34

Aggressive, fast-growing
Require combination chemotherapy
Can be cured

Question 35

What are the 2 commonest lymphomas?

Answer 35

Diffuse large B-cell lymphoma

Follicular lymphoma

Question 36

Is Diffuse large B-cell lymphoma low or high grade?

Answer 36

High grade

Question 37

Is follicular lymphoma low or high grade?

Answer 37

Low grade

Question 38

How are non Hodkin lymphoma treated?

Answer 38

Combination chemotherapy - anti-CD20 monoclonal antibody and chemotherapy

Question 39

Do men or women get Hodkin lymphoma more?

Answer 39

Men

Question 40

How is Hodgkin lymphoma treated?

Answer 40

Combination chemotherapy
\+/- radiotherapy
Monoclonal antibodies (anti-CD30)
Immunotherapy (checkpoint inhibitors)
PET scanning to assess response to treatment