Lymphoma Cases

Question 1

Lymphoma dx

Answer 1

PE (waldeyer's ring)
B sxs
CBC w/ differential, CMP, LDH, uric acid
Excisional biopsy needed (bone marrow, flow cytometry)
Staging imaging (CT/PET)
EF, PFTs
Hep B
Fertility counseling
HIV

Question 2

HL sxs

Answer 2

enlarging, rubbery, but not tender lymphadenopathy. Typically in the neck or chest.
Can be accompanied by 
Fatigue
FeverB
Weight lossB
Night sweatsB
Pain with EtOH
Symptoms of cytopenias
Hepatosplenomegaly
Pruritis

Question 3

_______ is a rare cancer, but in young people with cancer, it is very common.

Answer 3

Hodgkin Lymphoma

Question 4

malignant cell in HL

Answer 4

reed-sternberg

Question 5

Reed sternberg is a ___ cell, some are ____+

Answer 5

B cell

CD20+

Question 6

Bulk of HL tumor is

Answer 6

reactive tissue (background cells? = benign, but very prevalent)

Question 7

Classical HL types and marker

Answer 7

Nodular sclerosis (grades I and II)
Lymphocyte-rich classical
Mixed cellularity
Lymphocyte depleted

These are CD30+

Question 8

Non-classical HL type and marker

Answer 8

Nodular lymphocyte predominant

CD20+

Question 9

HL staging

Answer 9

Ann arbor staging:
I - single LN region
II - One side of diaphragm
III - both sides of diaphragm
IV - disseminated 

A - no systemic symptoms
B - fever, night sweats, weight loss

E - extralymphatic site
S - splenic site

Question 10

HL treatment

Answer 10

intent to cure!
Localized favorable risk: ABVD x 2 then restage, involved field radiation
Stage III/IV disease: ABVD x 2 (restage then okay) -> ABVDx4 +/- XRT
ABVD x2 (restage but refractory) then intense chemo BEACOPP, autologous transplant

Question 11

Late effects of therapy

Answer 11

Second malignancy (solid tumors ie lung and breast - risk continues to build, or leukemia - rare after 10-15 years)
Heart disease
Lung disease
Impaired fertility

Question 12

HL markers

a. CD15+, CD30+, EBER+, CD20-, CD79a-, CD45-
b. CD5-, CD23-, CD19+, CD20+
c. CD5+, CD10-, CD20+, CD23-, cyclin D1+
d. CD5+, CD19+, CD20(dim), CD22−, CD23+, CD79b−

Answer 12

a. CD15+, CD30+, EBER+, CD20-, CD79a-, CD45- = HL
b. normal B cell
c. mantle
D. CLL

Question 13

prognostics system for diffuse large b-cell lymphoma

Answer 13

IPI Prognostic factor:	
	Age over 60		
	Stage III/IV		
	ECOG PS 3 or 4 (more than ½ day resting)	
	Elevated LDH		
	Two or more extranodal sites		

Score 0 or 1: ‘Good’ with predicted 4-year progression-free survival of 94% and overall survival of 94%.
Score 2: ‘Intermediate’ with predicted 4-year progression-free survival of 80% and overall survival of 79%.
Score 3, 4 or 5: ‘Poor’ with predicted 4-year progression-free survival of 53% and overall survival of 55%

Question 14

prototype aggressive NHL for adults

Answer 14

Diffuse large b-cell lymphoma

Question 15

tx DLBL

Answer 15

R-CHOP

Question 16

most common indolent NHL

Answer 16

Follicular lymphoma

Question 17

cure follicular lymphoma?

Answer 17

Not curable

Question 18

follicular lymphoma cytogenetics

Answer 18

t(14;18)(q32;q21)
BCL-2–chromosome 18q21, inhibits apoptosis
IgH—chromosome 14q32
BCL-2 is translocated to chromosome 14, comes under control of IgH enhancer which leads to overexpression of BCL-2
But follicular lymphoma still primarily a diagnosis based on histology

Question 19

treatment of indolent NHL

Answer 19

Only when forced to:
‘Pushy nodes’
    Hydronephrosis
    Jaundice
    Dysphagia/stridor
Cytopenias (bone marrow failure)
B symptoms or fatigue

Question 20

Cyclin D1+

Answer 20

think mantle cell lymphoma

Question 21

tx mantle cell lymphoma

Answer 21

Indolent until it isn’t. Typically in advanced disease, use R-CHOP then autologous stem cell transplant or maintenance rituximab if not a transplant candidate.

Question 22

His example of markers in Mantle Cell lymphoma

Answer 22

CD20+ CD5+ BCL-2+ Cyclin D1

negative for CD3, CD10, CD21, CD23, BCL-6

Question 23

His example of markers in MALT lymphoma

Answer 23

CD20+, CD10-, CD5-, CD23-, cyclin D1-

Question 24

MALT lymphoma frequently with

Answer 24

H. pylori
treat H. pylori, get rid of Lymphoma sometimes
In other places (not stomach) too with other associated diseases

Question 25

Indolent NHLs

Answer 25

Follicular, MALT, marginal zone, SLL, etc (mycosis fungoides)

untreated can survive years
Not curable, wait to tx until sxs

Question 26

Aggressive NHLS

Answer 26

DLBC, mantle cell

untreated can survive months
sometimes curable
treat

Question 27

Very aggressive NHLs

Answer 27

Lymphoblastic, Burkitt, AID-related

untreated can survive weeks
curable in some
treat

Question 28

Hodgkin lymphoma

Answer 28

All types are variable survival in untreated, usually curable, treat

Question 29

solid organ transplant patients or other patients on immune suppression, particularly with the calcineurin inhibitors (tacrolimus & cyclosporine) or ATG, who develop lymphoma.

Answer 29

think of PTLPD (post-transplant lymphoproliferative disorder).
This is an EBV induced, polyclonal, expansion of B-cells. Why does it occur? The suppression of T-cells leads to a loss of suppression of B-cell proliferation.
It is treated by backing off on the immune suppression.

Question 30

T-cell lymphomas tested in the form of

Answer 30

mycosis fungoides

Question 31

MF/SS general principles

Answer 31

Not curable
Frequently relapse, but systemic involvement is uncommon
Treatment is UV light, radiation, topical or systemic

Question 32

systemic T cell lymphoma

Answer 32

These behave badly
Again, CHOP is often the first line treatment
No rituximab!
If CD 30+, then brentuximab vedotin as targeted therapy

Question 33

CD30+ monoclonal ab

Answer 33

brentuximab vedotin