Lymphoma - Kebbekus

Question 1

What is important to examine when thinking about a lymphoma diagnosis?

Answer 1

Physical exam
check all lymph node chains
B symptoms
Weight loss (>10%)
Drenching night sweats
Fever (don't make sense)
CBC with differential
Metabolic panel
LDH
HIV testing
Uric acid (purines from killed cells)
prevent kidney disease
Excisional biopsy
If cancer is the issue, tissue is the answer
Bone marrow
Flow cytometry
Staging imaging (CT/PET)

Question 2

What characterizes Hodgkin’s Lymphoma?

Answer 2

Malignant cell = Reed-Sternberg cell
Now recognized as a B-cell origin
some are CD20+
Bulk of the tumor is reactive tissue
Spreads contiguously from node to node

Question 3

What are the symptoms of Hodgkins’ Lymphoma?

Answer 3

Enlarging, rubbery, but not tender lymphadenopathy
typically in neck or chest
Accompanied by:
fatigue
feverB
weight lossB
night sweatsB
Pain with EtOH
Symptoms of cytopenias
Hepatosplenomegaly

Question 4

How common is Hodgkin’s Lymphoma?

Answer 4

Rare cancer
Common in young people with cancer
1 in 5 types of cancer for young people

Question 5

What is the best chemotherapy treatment for Hodgkin’s Lymphoma?

Answer 5

ABVD
Adriamycin (cardiotoxicity)
Bleomycin (lung toxicity)
Vinblastine (numbess)
Dacarbazine

Question 6

What are the stages of Lymphomas?

Answer 6

Ann Arbor Staging
I: single lymph node region
II: One side of diaphragm
III: Both sides of diaphragm
IV: Disseminated
effusions
liver & spleen
bone marrow

Question 7

How is localized favorable risk HL disease treated with the intent to cure?

Answer 7

ABVD x2 (restage)
Involved field radiation

Question 8

How is Stage III/IV HL Disease treated?

Answer 8

ABVD
BEACOPP
Autologous transplant

Question 9

What are the late effects of chemotherapy in HL?

Answer 9

Second malignancy
Heart disease (MI, CAD)
Lung disease
Impaired fertility

Question 10

What markers do you look for in Lymphoma?

Answer 10

CD5-
CD19+
CD20+
CD23-

Question 11

What sytem do you use to classify Non-Hodgkin’s Lymphoma?

Answer 11

IPI Scoring System
score 0-5
0-1 = cure 
2 = cure most
3-5 = cure about half
Prognostic factor: 
  Age over 60 
  Stage III/IV 
  ECOG PS 3 or 4 (more than ½ day resting) 
level of physical health
  Elevated LDH 
  Two or more extranodal sites

Question 12

What characterizes Diffuse Large B-cell Lymphoma?

Treatment?

Answer 12

Prototype aggressive NHL
Accounts for 40% of lymphomas
Average age of onset is 67
75.3% of cases are above the age of 55
If left untreated it is quickly fatal
Treated with CHOP (more intense regimens have not been shown to improve overall survival, 1993)
The addition of rituximab (anti-CD20) in 1997 was a difference maker: R-CHOP

Question 13

What translocation is associated with Follicular Lymphoma?

Answer 13

t(14;18)
Bcl-2 is translocated to chromosome 14
comes under control of IgH enhancer which leads to overexpression of BCL-2

Question 14

Is follicular lymphoma cureable?

Answer 14

Typically (over 80%!) advanced stage at presentation
Often asymptomatic
FL not curable with chemotherapy
Most common indolent NHL

Question 15

What is the survival rate in Follicular Lymphoma?

Answer 15

Stage I-II
50% long term (10-20 yrs) disease free survival with radiotherapy alone (?cure?)
​Stage III-IV
Remission rate
70-85% with chemo
50-70% with rituximab
70-90% with chemo + rituximab
Cure rate 0%
Median survival 10-12 years, longer in younger pts

Question 16

What kind of lymphoma expresses cyclinD1?

Answer 16

Mantle Cell Lymphoma

Question 17

What is MALT Lymphoma associated with?

Answer 17

H. pylori

eradicate infection first (often cures MALT Lymphoma)

Question 18

What type of lymphoma do solid organ transplant patients get?

Answer 18

Post-transplant Lymphoproliferative Disorder
poly-clonal, expansion of B-cells
The suppression of T-cells (immunosuppressives to prevent organ rejection) leads to a loss of suppression of B-cell proliferation

Question 19

What are the general principles of T-cell lymphomas?

Answer 19

Not curable
Frequently relapse, but systemic involvement is uncommon
Treatment is UV light, radiation, topical or systemic
CHOP is first line treatment
If CD 30+, then brentuximab vedotin as targeted therapy