Lymphoid Malignancies

Question 1

Most common lymphoid malignancies?

Answer 1

NHL (high grade)
NHL (low grade)
Hodgkin’s lymphoma
CLL
ALL
MM

Question 2

What’s the most common high and low grade NHLs?

Answer 2

High grade = Diffuse Large B Cell Lymphoma

Low Grade = Follicular Lymphoma

Question 3

How would you expect to see a lymphoma?

Answer 3

Lymphadenopathy & Hepatosplenomegaly

Can also have extranodal disease, bone marrow involvement and B symptoms (Fever, night sweats & weight loss)

Question 4

What tests would you do on a suspected Lymphoma?

Answer 4

Biopsy the node
Aspirate bone marrow
CT scan

Question 5

What’s the difference between high and low grade NH lymphomas?

Answer 5

Low grade = often asymptomatic, not curable but treatable

High grade = Aggressive & fast growing, needs combo-chemo but curable

Question 6

What treatment is used for NH lymphomas?

Answer 6

Combination Chemo (Anti-CD20 monoclonal Abs + Chemo)

Question 7

Who gets Hodgkin’s LYmphoma?

Answer 7

It peaks at 15-35yrs then later in life (2nd peak ass with EBV)

More often men.

familial risk and geographical clustering suggest a genetic pattern

Question 8

How would you treat a Hodgkin’s Lymphoma?

Answer 8

Combination Chemo (ABVD)
+/- RT
Anti-CD30 MAB
Immunotherapy

Question 9

What would a patient with CLL look like?

Answer 9

Often asymptomatic but when they get problems:
- progressive Bone marrow failure
- Lymphadenopathy
- ~ Splenomegaly, fever & sweats
- Less often hepatomegaly, infections & weight loss

Also immune paresis (no immunoglobulin production)
And Autoimmune Haemolytic Anaemia or ITP

Question 10

How would you stage a CLL patient?

Answer 10

Binet Staging:
A - some nodes
B - more nodes
C - other symptoms or autoimmune

Question 11

When would you treat someone with CLL?

Answer 11

Only if there’s signs that it’s worsening:
- Progressive marrow failure
- Massive lymphadenopathy
- Progressive Splenomegaly
- Lymphocyte doubling time <6months
- Systemic Symptoms
- Autoimmune Cytopenias

Question 12

How do we treat a case of CLL?

Answer 12

Often just watch and wait. If it’s stable they may be better off without treatment

Cytotoxic Chemo e.g. Fludarabine
Monoclonal Abs e.g. Rituximab
Tyrosine Kinase Inhibitors e.g. Ibrutinib

Question 13

What factors would indicate a poor prognosis for CLL?

Answer 13

• Stage B & C
• Atypical lymphocyte morphology
• Rapid lymphocyte doubling time (<12month)
• Loss/mutation of p53
• CD38+ expression
• Unmutated IgVH gene

Question 14

What is required to diagnose cLL?

Answer 14

1) Blood >5x10^9/L lymphocytes
2) Bone Marrow >30% lymphocytes
3) Characteristic Immunophenotyping

Question 15

Who gets ALL?

Answer 15

75% are in kids <6

Question 16

how does ALL present?

Answer 16

Acute (2-3wks) h/o:
- Bone Marrow Failure (Anaemia, bleeds & infections)
- Bone/joint pain
- Infections
- Sweats

Question 17

What do we need to diagnose ALL?

Answer 17

Do a FBC to show low haemonoglobin, low platelets, low neutrophils and high WCC

Confirm with a Marrow biopsy showing >20% lymphoblasts

Question 18

What can we then treat ALL with?

Answer 18

Multiagent Intensive chemo (Induction, Consolidation & Maintenance)

Allogenic stem cell transplant

There are new T cell immunotherapies

Question 19

Poor prognostic factors for aLL?

Answer 19

Older
Higher WCC
Immunophenotype
Certain genetics
Slow/poor response to treatment

Question 20

What are the main T cell immunotherapies for ALL?

Answer 20

BiTe molecules (Bispecific T cell engagers)

CAR T cells (Chimeric Antigen Receptor T Cells)

Question 21

Potential side effects of ALL T-cell immunotherapies?

Answer 21

Cytokine Release Syndrome i.e. fever, hypotension & SOB

Neurotoxicity - Confusion with normal conscious level, seizures, headache, focal neurology & coma

Question 22

Prognosis for ALL?

Answer 22

Adults have a roughly 50% cure rate
Kids more like 90%