Hodgkin's Lymphoma

Question 1

why is the Ann Arbor Staging important in HL

Answer 1

bc it spreads continguously

Question 2

what is the malignant hallmark for HL

Answer 2

Reed-Sternberg cells

Question 3

describe the clinical behavior of HL

Answer 3

very heterogenous

indolent to fulminant disease

Question 4

is HL curable?

Answer 4

yes - 80%

can irradiate the suckers

Question 5

which HL has the best prognosis?

Answer 5

lymphnocyte predominant

10+ years median survival

Question 6

whcih is the worst prognosis?

Answer 6

lymphocyte depleted

0.3 years median survival

Question 7

is the etiology known?

Answer 7

nope

Question 8

which virus is associated with HL

Answer 8

EBV about 50%

Question 9

What does HL cause in advanced stage

Answer 9

depression of cell mediated immunity

Question 10

At what age does HL occur?

Answer 10

bi-modal age distribution
first peak - 20
second peak - .60

Question 11

What other disease is bi-modal age distribution seen in?

I dk if this is correct!

Answer 11

ALL –> peds is most common

usually peaks early then theres a steady incline into adulthood

Question 12

what is most common presentation of Hodgkins Disease (HD)

Answer 12

painless enlargement of cervical and/or supraclavicular LN

Question 13

What can be detected using a chest xray

Answer 13

anterior mediastinal mass

Question 14

what are the systemic symptoms of HD

Answer 14

unexplained fevers, drenching night sweats, weight loss > 10% body weight

Question 15

what is considered a risk factor for HD

Answer 15

age and the stage they are in

Question 16

What are the disease characteristics of HD

Answer 16

UNIFOCAL in origin

Predictable spread - adjacent lymph nodes

Question 17

What are the disease characteristic exceptions of HD

Answer 17

1. retrograde spread from cervical to retroperitoneal LN –> via the thoracic duct
2. spleen –> liver
3. vascular invasion is rare

Question 18

How do you diagnose HD?

Answer 18

biopsy of invovled tissue
-especially at relapse

-knowing the hx of LN in HL is important bc you need to know if its a residual/scar tissue and not new

Question 19

if HL is limited disease, what treatment method do you use?

Answer 19

radiation therapy

Question 20

if Hl is widespread/extranodal invovlement, treatment?

Answer 20

systemic chemo

Question 21

anatomical staging is important in HL bc?

Answer 21

critical for making treatment decisions and it defines the limits of the disease

Question 22

What is the Ann Arbor Staging?

Answer 22

I Single lymph node (LN region (I) or a single extranodal (localized) organ or site (IE)

II Two ore more lymph nodes on the same side of the diaphram (II) or localized involvement of an extralymphatic organ or site of the diaphragm (IIE)

III Lymph node regions on both sides of the diaphragm (III) which may also involve the spleen (IIS) or local involvement of an extralymphatic organ or site(IIIE) or both (IIISE)

IV Diffuse or disseminated involvement or one or more extralymphatic organs without associated lymph node involvement

Question 23

What was the historical treatment for HL

Answer 23

MOPP
M - Mechlorethamine
O - Oncovin (Vincristine)
P -  Procarbazine
P -  Prednisone

Question 24

What is the treatment regimen used now

Answer 24

ABVD
A - Adriamycin
B - Bleomycin
V - Vinblastine
D - Dacarbazine

Question 25

What is the trend in HL treatment?

Answer 25

to get away from chemo

Question 26

what are the reasons why ABVD is better than MOPP

Answer 26

MOPP is very leukemagenic – can cause leukemia in the long term
MOPP causes sterility

ABVD does the opposite of MOPP

Question 27

What do you do for recurrent HL

Answer 27

Post XRT almost always just outside of radiation field

Post chemotherapy are systemic and are best treated with high-dose therapy and autologous PBSCT

**ADC – Brentuximab vedotin

With extensive disease and bone marrow involvement, allogeneic or MUD BMT is preferred

Lymphocyte Predom – Role of rituximab

Question 28

What cell does ADC target

Answer 28

CD30+

Question 29

What is Brentuximab vedotin MOA?

Answer 29

bind with CD30+ on HL cell surface –> then forms a complex with CD30 and enters into the cell –> once inside, ADC chemo component is relased and kills the cancer cell