Lymphoma 2

Question 1

What is the presentation of lymphoma?

Answer 1

Painless progressive lymphadenopathy
Infiltrate/impair an organ system
Recurrent infections
Constitutional symptoms
Coincidental

Question 2

How may painless lymphadenopathy be detected?

Answer 2

Palpable node

Extrinsic compression of any ‘tube’ e.g. ureter, bile duct, large blood vessel, bowel, trachea, oesophagus

Question 3

What are the diagnosis and staging steps of lymphoma?

Answer 3

Histological diagnosis
Anatomical stage (scans)
Blood tests (baseline, give effect of lymphoma on the patient)

Question 4

What is the key malignant cell in Hodgkin Lymphoma?

Answer 4

Reed Sternberg cells

Question 5

What are the more common patient characteristics of Hodgkin lymphoma?

Answer 5

Bimodal age incidence - age 20-29, smaller peak affecting >60
More common in males

Question 6

What are the subcategories of classical Hodgkin Lymphoma?

Answer 6

Nodular sclerosing
Mixed cellularity
Lymphocyte rich/depleted (rare)

Question 7

What is stage I for HL?

Answer 7

One group of nodes

Question 8

What is stage II for HL?

Answer 8

> 1 group of nodes same side of diaphragm

Question 9

What is stage III for HL?

Answer 9

Nodes above and below the diaphragm

Question 10

What is stage IV for HL?

Answer 10

Extranodal spread

Question 11

What does suffix A/B mean in HL staging?

Answer 11

A is none, B is any of
Fever
Unexplained weight-loss
Night sweats

Question 12

What are the characteristics of sclerosing subtype of HL?

Answer 12

Young women (>men) 20-29yrs
Neck nodes and mediastinal mass
May have B symptoms
Needs a tissue diagnosis

Question 13

What is the treatment of HL?

Answer 13

Chemotherapy
ABVD 2-6 cycles
+/- radiotherapy

Question 14

What is the role of radiotherapy in HL?

Answer 14

Results in low/negligible risk of relapse within field

Question 15

What is Non-Hodgkin Lymphoma?

Answer 15

Neoplastic proliferation of lymphoid cells

Question 16

What are the steps of management of Non Hodgkin Lymphoma?

Answer 16

Stage disease
Prognostic markers
Plant therapy

Question 17

How is NHL staged?

Answer 17

CT scan
PET scan (indicated in aggressive lymphomas)
BM biopsy
Lumbar puncture (if risk of CNS involvement

Question 18

What are the two most common types of non Hodgkin Lymphoma?

Answer 18

Follicular lymphoma

Diffuse large B cell lymphoma

Question 19

What is the clinical behaviour of Burkitt Lymphoma and T or B cell lymphoblastic leukaemia/lymphoma

Answer 19

Very aggressive

Question 20

What is the clinical behaviour of Diffuse large B cell and mantle cell lymphoma?

Answer 20

Aggressive

Question 21

What is the clinical behaviour of follicular, small lymphocytic/CLL and mucosa associated (MALT)?

Answer 21

Indolent

Question 22

What is the prognosis of Diffuse Large B cell NHL determined by?

Answer 22

IPI (International Prognostic Index)
Age
Stage (Ann Arbor)
LDH
Extra-nodal disease sites
ECOG performance status

Question 23

What is the genetic association of follicular NHL?

Answer 23

t(14;18) which results in over expression of bcl2 an anti-apoptosis protein

Question 24

What is the median survival of follicular NHL?

Answer 24

12-15yrs

Question 25

What are the management options for follicular lymphoma?

Answer 25

Watch and wait | Treatment

Question 26

What would be indications for treatment while watching and waiting follicular NHL?

Answer 26

Nodal extrinsic compression: bowel, Bile duct, ureter, vena cava Massive painful nodes Recurrent infections

Question 27

What is the treatment for follicular NHL?

Answer 27

Combination immuno-chemotherapy

Question 28

What is MZL?

Answer 28

Marginal Zone Lymphoma involving extra-nodal lymphoid tissue (e.g. Gastric mucosa-associated lymphoid tissue MALT/H. Pylori, Parotic MZL/Sjogren syndrome)

Question 29

What is enteropathy associated T cell lymphoma (EATL)?

Answer 29

T cell NHL seen in patients with Coeliac disease Has an aggressive clinical course

Question 30

What is the presentation and clinical course of EATL?

Answer 30

Abdominal pain, obstruction perforation, GI bleeding Malabsorption Systemic symptoms Responds poorly to chemo, generally fatal Aim to prevent (strict adherence to Gluten Free diet)

Question 31

What is chronic lymphocytic leukaemia?

Answer 31

Proliferation of mature B-lymphocytes

Question 32

What are the laboratory findings in CLL?

Answer 32

``` Lymphocytosis between 5 and 300 x 10^9/l Smear cells Normocytic normochromic anaemia Thrombocytopenia Bone marrow lymphocytic replacement of normal marrow elements ```

Question 33

What are the different progressions of CLL?

Answer 33

Never progress Progress but respond to treatment Progress, require multiple lines of treatment --> death

Question 34

What is a genetic indicator of poor prognosis in CLL?

Answer 34

Deletion of 17p

Question 35

What are the clinical issues in CLL?

Answer 35

Increased risk of infection Bone marrow failure Lymphadenopathy +/- splenomegaly, lymphocytosis Transform to high grade lymphoma (Richter Transformation) Auto-immune complications

Question 36

What are the indications to treat CLL?

Answer 36

``` Progressive lymphocytosis Progressive marrow failure Massive or progressive lymphadenopathy / splenomegaly Systemic symptoms Autoimmune cytopenias ```

Question 37

What are the treatment options for CLL?

Answer 37

``` Combination Immuno-chemotherapy Targeted therapy (BTK inhibitor, BCL2 inhibitor) Cellular therapy for relapsed high risk cases ```

Question 38

Give an example of BCR Kinase inhibitors

Answer 38

Ibrutinib (BTK) | Idelalisib (PI3K)

Question 39

Give an example of BCL2 inhibitors

Answer 39

Venetoclax

Question 40

Give an example of experimental Cell based therapies

Answer 40

Chimaeric Antigen Receptor T cells (CAR-T)