INDOLENT LYMPHOMAS

Question 1

what is lymphoproliferative disease?

Answer 1

neoplastic, clonal proliferation of lymphoid cells
- a cancer of white blood cells

Question 2

what does lymphoproliferative disease usually affect?

Answer 2

lymph nodes

Question 3

where can lymphoproliferative disease be?

Answer 3

Can be Extranodal – Bone Marrow / Liver / Spleen / Anywhere

Question 4

what are the two types of non Hodgkins lymphoma?

Answer 4

agressive
indolent

Question 5

indolent non H lymphoma

Answer 5

slowly growing and advanced at presentation
don’t require treatment
usually incurable

Question 6

aggressive non H lymphoma

Answer 6

grow faster r
chemo sensitive
curable

Question 7

H lymphoma

Answer 7

tends to be in younger people

Question 8

how is NHL classified?

Answer 8

B cell - 90%
T cell- 10%
NK cell- <1%

Question 9

what are the different subtypes of indolent lymphoma?

Answer 9

• follicular
• marginal one lymphoma
  -mantle cell lymphoma
• CLL

Question 10

what does the cause of dead tend to be with people who have indolent lymphoma?

Answer 10

-Cause of death usually related to Lymphoma and not anything else

• Relapse/Refractory disease;
  -High grade transformation (Richter’s)*

Question 11

what is the cause of indolent lymphomas? ( aetiology)

Answer 11

• most cases is unknown

Question 12

what are the Risk factors for indolent lymphomas?

Answer 12

• primary immunodeficiency-
  e.g. Wiscott-Aldrich Syndrome; Common Variable Immunodeficiency
• secondary immunodeficiency-
  e.g. HIV; Recipients of Transplant
• infection
  e.g. EBV; HTLV-1; Helicobacter Pylori
• autoimmune disorders

Question 13

describe the clinical presentation of someone with indolent lymphomas

Answer 13

-painless lymphadenopathy- ( lump)

-Association with B-Symptoms – Fevers, Night Sweats and Weight Loss

-?Bone Marrow involvement – ?Leukaemic component- low blood count

-Autoimmune Phenomena

-Compression Syndromes

-Organ Involvement e.g. Skin

Question 14

what investigations would you do for indolent lymphomas?

Answer 14

Lymph Node Biopsy – Core Needle Biopsy/ Excision Node Biopsy (NOT FNA)

?Bone Marrow Biopsy

Staging
lugano (Update on Ann Arbor) Staging Classification typical for most Indolent lymphomas
Requires Imaging – CT Neck/Thorax/Abdomen/Pelvis or PET-CT

Bloods
Bone Marrow

Question 15

what treatment would you do for indolent lymphomas? ( name them)

Answer 15

-Watch and Wait / Active Surveillance
-Radiotherapy
-Chemoimmunotherapy +/- maintenance
-Small molecules inhibitors / Novel therapies
-Bi-Specific T-Cell engaging Antibodies and Chimeric Antigen Receptor T Cells

Question 16

describe the Watch and Wait / Active Surveillance treatment method

Answer 16

-Asymptomatic, advanced stage patients

-Follow up regularly

-No compromise in overall survival

Question 17

describe radiotherapy as a treatment method

Answer 17

• Local Control (Palliative)
• potentially can be Curative

Question 18

describe Chemoimmunotherapy +/- maintenance as a treatment method

Answer 18

-Alkylating agents e.g. Chlorambucil, Bendamustine, Cyclophosphamide

• Anthracyclines e.g. Doxorubicin - Vina Alkaloids e.g. Vincristine
• Corticosteroids
• Combinations

-Monoclonal Antibodies
-Anti-CD20 e.g. Rituximab; Obinutuzumab

- Maintenance therapy – Follicular Lymphoma; Mantle Cell Lymphoma

Question 19

describe Bi-Specific T-Cell engaging Antibodies and Chimeric Antigen Receptor T Cells as a treatment method

Answer 19

Emerging therapies in indolent lymphoma

CAR-T NICE Approved for BTKi failures in Mantle Cell Lymphoma

Mosunetuzumab – Trials in FL / MCL / DLBCL

Question 20

describe Small molecules inhibitors / Novel therapies as a treatment method

Answer 20

Brutons Tyrosine Kinase Inhibitors

BCL2 Inhibitors

IMiDs