lymphoid malignancy

Question 1

lymphoma

Answer 1

lymphoid origin
can present with enlarged lymph nodes (lymphadenopathy)
or
extranodal involvement
or 
bone marrow involvement
systemic B symptoms

Question 2

diagnosis

Answer 2

biopsy tells us what type
clinical exam and imaging tells staging
-info of prognosis

Question 3

therapy

Answer 3

never surgical resection

Question 4

hodgkin lymphoma

Answer 4

specific disease

Question 5

non hodgkin

Answer 5

broad term/everything else

Question 6

lymphoproliferative disorders

Answer 6

ALL
CLL
Hodgkin
Non-hodgkin
- high grade (difffuse large B cell lymphoma)
- low grade (follicular, marginal zone)

Question 7

ALL

Answer 7

disorder of lymphoid progenitor cells
Leukaemia = No differentiation. Instead, rapid, uncontrolled growth and accumulation
Usually in bone marrow but they can go anywhere
-75-90% in children under 6
B cell lineage mostly affected
present with 2-3 week of bone marrow failure or bone/joint pain

Question 8

typical ALL case

Answer 8

17yr old male
1 month impaired vision(both eyes)
half stone weight loss
breathless on minimal exertion
hb and platelets low
white cell very high

Question 9

Characteristics

Answer 9

large cells
express CD19- all B cells
CD34,TDT-markers of very early immature cells

Question 10

treatment

Answer 10

standard
-induction chemotherapy to obtain remission
-consolidation therapy
-CNS directed treatment
-maintenance treatment for 18 months
transplantation

Question 11

ALL treatment

Answer 11

Newer therapies

1) Bi specific T cell engagers?
2) Chimeric antigen receptor T cells
- Healthy T cells harvested
- transfected to express a specific T cell receptor expressed on leukemia cells (CD19)

Question 12

T cell immunotherapy side effects

not in exam

Answer 12

Cytokine release syndrome -fever, hypotension, dyspnoea
neurotoxicity- confusion
seizure, headache, coma

Question 13

Poor risk factors ALL

Answer 13

increasing age
white count
cytogenetics
t(9,22); t(4,11)
poor response

Question 14

summary ALL

Answer 14

bone marrow failure +/- raised white cell count
Bone pain, infection, sweats
Treated with multiagent intensive chemo +/- allogeneic stem cell transplant

Question 15

CLL

Answer 15

the abnormal cells are mature – they usually resemble normal, well-behaved lymphocytes
Grow slowly (or not at all)
Classic example of a low-grade condition
Carry many of the normal markers that B lymphocytes have

Requires a lymphocyte count of > 5 (normal is < 4)

Question 16

CLL continued

Answer 16

Commonest leukemia
occasionally familial
2 makes: 1 female
often asymptomatic
-bone marrow failure
-lymphadenopathy
-splenomegaly 
fever and sweats
Less common findings:
hepatomegaly
infections weight loss

Question 17

associated findings

Answer 17

immune paresis (loss of Ig production)
Haemolytic anaemia

Question 18

CLL staging

Answer 18

Stage A
<3 lymph nodes
median survival same as age matched controls
stage B 3 or more lymph nodes
Stage C
Stage B- anaemia or thrombocytopenia

Question 19

indications for treatment

Answer 19

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

Question 20

treatment

Answer 20

incurable
low grade
watch and wait
cytotoxic chemotherapy
monoclonal antibodies
eg Rituximab
novel agents

Question 21

poor prognostic marker

Answer 21

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 22

what is an adverse prognostic factor

Answer 22

loss/mutation of P53

Question 23

ann arbour staging system

Answer 23

I-IV

Question 24

B cells

Answer 24

majority of leukemias

Question 25

high grade lymphoma

Answer 25

Aggressive, fast-growing
Require combination chemotherapy
Can be cured

Question 26

low grade

Answer 26

Indolent, often asymptomatic

responds to chemotherapy but incurable

Question 27

non hodgkin lymphoma

Answer 27

Diffuse large B-cell lymphoma
Commonest subtype of lymphoma (of any kind)
High-grade lymphoma
Follicular lymphoma
2nd commonest subtype of lymphoma
Low-grade lymphoma
Like CLL, leave alone if not causing problems – “watch and wait”
Both are treated with combination chemotherapy – typically anti-CD20 monoclonal antibody + chemo

Question 28

hodgkin lymphoma

Answer 28

30% of all lymphomas
-association with epstein barr virus; familial and geographical clustering

Treatment (dont need to know)
Combination chemotherapy (ABVD)
\+/- radiotherapy
Monoclonal antibodies (anti-CD30)
Immunotherapy (checkpoint inhibitors)
1st peak at 15-35y (not associated to virus)
2nd peak later in life

PET scanning central to assessment of response to treatment
associated to immunity to fight viruses

Question 29

features

Answer 29

hodgkin lymphoma

contiguos lymph nodes