Haem: Lymphoma

Question 1

Define lymphoma

2 types lymphoma

Answer 1

Neoplastic tumour of lymphoid tissue (LN +/- BM, blood)

Hodgkin’s (20%), Non-Hodgkin’s (80%)

Question 2

M>F
peak incidence 20-29 and >60 y/o
EBV associated 
spreads contiguously 
2 subtypes:

Answer 2

Hodgkin’s lymphoma

• classical HL
• nodular lymphocyte predominant HL

Question 3

Asymmetrical painless lymphadenopathy
+/- obstructive Sx
B symptoms: fever, wt loss, nigh sweats
Pain in nodes after alcohol

Answer 3

Hodgkin’s lymphoma

Question 4

Reed-Sternberg cells
Owl eye nuclei

Ix = LN or BM biopsy. Cells stain CD15, CD30 +ve

Answer 4

Hodgkin’s lymphoma

Question 5

Describe Ann Arbor staging (for HL and NHL)

Stage 1 - 4 and A or B

Answer 5

think DIAPHRAGM
Stage 1 = one LN on one side of diaphragm
Stage 2 = 2+ LN on one side of diaphragm
Stage 3 = 2+ LN on both sides of diaphragm
Stage 4 = extranodal (liver, BM)

A = no B Sx, but can have pain 
B = B Sx

Question 6

Prognosis for HL
Treatment?

Worse prognosis if….

Answer 6

Prognosis excellent, cure rate 50-90%

Tx = combination chemo (ABVD: Adriamycin, bleomycin, vinblastine, dacarbazine)
+/- Radiotherapy (high risk of secondary breastca 25%, or cardiovascular events)
Intensive chemo + autologous SCT on relapsed pts

Worse prognosis if older and low WCC on histology

Question 7

Painless lymphadenopathy
+/- obstructive Sx and B Sx
no pain in nodes after alcohol

Staging = Ann Arbor

Incidence rising

Answer 7

Non-Hodgkin’s lymphoma (80% of lymphomas)

Question 8

3 Examples high grade NHL

Answer 8

Burkitt’s
Diffuse Large B-cell lymphoma
Mantle Cell

Question 9

3 Examples low grade NHL

Answer 9

Follicular NHL
Marginal XOne
Small Lymphocytic

Question 10

High grade (aggressive)
B cell lymphoma
t(8;14) translocation
c-myc oncogene ++

histology: starry sky appearance

Curable

Answer 10

Burkitt’s NHL (Bc)

Tx = rituximab (anti CD20) or SCT

Question 11

EBV associated B cell lymphoma
Africa
Jaw involvement and abdo masses

Answer 11

Endemic Burkitt’s NHL (Bc)

NB. HL also associated with EBV

Question 12

Common aggressive B cell lymphoma
middle age/elderly
can progress from CLL (Richter’s)
Curative in 50%

Histology: sheets of large lymphoid cells

p53+ = poor prognosis

Answer 12

Diffuse large B cell lymphoma
(aggressive NHL)

Ritxumab-CHOP or auto SCT = Tx

Question 13

M> F
middle age 
aggressive B cell NHL 
disseminated at presentation 
median survival 3-5 years 

t (11;14) translocation
cyclin D1 ++

Histology: angular nuclei

Answer 13

Mantle cell NHL (Bc)

Ritxumab-CHOP or auto SCT = Tx

Question 14

t(14;18)
high bcl12 expression

Histology: follicular pattern/nodular appearance

median survival 12-15y
indolent NHL

Answer 14

Follicular NHL (Bc)

Tx = watch and wait, if Sx give Rituximab

Question 15

Chronic antigen stimulation
Middle aged
marginal Zone Bc NHL
No B Sx

H pylori ->
Sjogrens->
Hashimotos ->

Answer 15

H pylori -> Gastric MALT lymphoma
(Tx = Amox/Clarith/Omep), +/- Chemo)

Sjogrens -> parotid MALT lymphoma

Hashimotos -> thyroid MALT lymphoma

ALL MALT lymphomas = Bc indolent NHL

Question 16

high WCC and smear cells 
\+/- BM failure 
CD19 and 22+
CD5+
Tdt -ve 

Bad prognosis: CD38+, IgH UN mutated, tp53/17p deletion

Caucasian/Western
Elderly
can progress to Richter’s (DLBCL in 1%)

Answer 16

Chronic lymphocytic leukaemia
(Mature Bc NHL)

Tx = vaccination (flu and pneumococcus) and ABx/Antivirals

Question 17

Tdt +ve Bc

Answer 17

aggressive Bc lymphoblastic lymphoma

NHL

Question 18

Caribbean and Japanese
HTLV-1 associated
aggressibe

Answer 18

adult Tc leukaemia/lymphoma

Question 19

Associated with long-standing Coeliac disease

Answer 19

Enteropathy-associated Tc lymphoma

Question 20

Associated with mycosis fungoides

Answer 20

Cutaneous Tc lymphoma