Lymphoid Malignancy

Question 1

What are the possible presentations of a lymphoma?

Answer 1

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

Question 2

How are lymphomas classified?

Answer 2

Hodgkins lymphoma

Non-hodgkins lymphoma (covers >70 subtypes)

Question 3

What test can identify the lymphoma subtype?

Answer 3

Biopsy

Question 4

What is acute lymphoblastic leukaemia?

Answer 4

A cancerous disorder of lymphoid progenitor cells

Question 5

How does ALL present?

Answer 5

3/4 of cases in children

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

Question 6

What are the characteristics of ALL cells?

Answer 6

Large, aggressive and fast growing

Express CD19, CD34 and TDT

Question 7

How is ALL treated?

Answer 7

Induction chemotherapy to obtain remission
Consolidation therapy
CNS directed treatment
Maintenance treatment for 18 months (remission will come about quickly and blood count will normalise but relapses will occur unless treatment is given for 18-24 months)
Stem cell transplantation (if high risk- adult)

Question 8

What are the poor prognostic factors for ALL?

Answer 8

Increasing age
Increased white cell count
Cytogenetics/molecular genetics, Philadelphia chromosome
Slow/poor response to treatment

Question 9

What is chronic lymphocytic leukaemia?

Answer 9

Cancerous disorder of mature lymphocytes. It is a low-grade condition that grows slowly and cells act very similarly to normal B cells

Question 10

How common is chronic lymphocytic leukaemia?

Answer 10

Commonest leukaemia worldwide

M:F = 2:1

Question 11

What are some frequent findings in CLL?

Answer 11

Patients often asymptomatic
Bone marrow failure (anaemia, thrombocytopenia)
Lymphadenopathy 
Splenomegaly (30%) 
Fever and sweats (< 25%)
Hepatomegaly (less common) 
Weight loss (less common)
Infections (less common)
Immune paresis and haemolytic anaemia can occur with CLL

Question 12

How is CLL staged?

Answer 12

Stage A- <3 lymph node areas
Stage B- 3 or more lymph node areas
Stage C- Stage B + anaemia or thrombocytopenia

Question 13

What is the prognosis for each stage of CLL?

Answer 13

Stage A- Same as age matched controls
Stage B- ~8 years
Stage C- ~6 years

Question 14

What are the indications for treatment in CLL?

Answer 14

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

Question 15

How is CLL treated?

Answer 15

Often nothing
Cytotoxic chemotherapy
Monoclonal antibodies
Novel agents- bruton tyrosine kinase inhibitors, P13K inhibitors, BCL-2 inhibitors

Question 16

What are the poor prognostic factors for CLL?

Answer 16

Advanced disease (Binet stage B or C)
Atypical lymphocyte morphology
Rapid lymphocyte doubling time (<12 mth)
CD 38+ expression
Loss/mutation p53; del 11q23 (ATM gene)
Unmutated IgVH gene status

Question 17

How is lymphoma staged with the Ann Arbour system?

Answer 17

1- One lymph node group
2- Two or more lymph node groups on one side of the diaphragm
3- Lymph node involvement on both sides of the diaphragm
4- Disseminated disease with involvement of extranodal sites (commonly marrow, liver, abdominal wall)

A- Absence of B symptoms
B- Fever, night sweats, weight loss

Question 18

What are the commonest subtypes of non-Hodgkin lymphoma?

Answer 18

Diffuse large B-cell lymphoma- high grade

Follicular lymphoma- low grade

Question 19

How are diffuse large B-cell and follicular lymphomas treated?

Answer 19

Watch and wait with follicular lymphoma if not causing problems
Combination chemotherapy (chemotherapy + monoclonal antibody- usually rituximab)

Question 20

What populations are most at risk of Hodgkin lymphoma?

Answer 20

Peak ages of Hodgkin lymphoma at 15-35 (not EBV related) and then again later in life (EBV associated).
M:F = 2:1

Question 21

How is Hodgkin lymphoma treated?

Answer 21

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