16 - Lymphoproliferative Disorders 2

Question 1

mycosis fungoides

Answer 1

indolent T cell (CD4) NHL
affinity for skin
can be tx w/ XRT, UV light, topicals or systemic chemo
leukemic form is know as sezary syndrome
cerebriform nuclei

Question 2

leukemic form of mycosis fungoides

Answer 2

Sezary syndrome

Question 3

anaplastic large cell lymphoma

Answer 3

aggressive T cell NHL
CD30 pos
skin often involved

Question 4

translocation associated with good prognosis anaplastic large cell lymphoma

Answer 4

t(2;5) - express of ALK (anaplastic lymphoma kinase)

Question 5

lymphoblastic lymphoma

Answer 5

highly aggressive T cell NHL
male adolescents
mediastinal mass
basically lymphoma form of ALL

Question 6

adult T cell leukemia/lymphoma

Answer 6

highly aggressive
assoc w/ HTLV1
mostly seen in Japan/Caribbean
frequent hypercalcemia
CD4 cells

Question 7

prognosis for hodgkin dz

Answer 7

highly treatable and curable, even when disseminated

Question 8

key cell of hodgkin dz

Answer 8

reed sternberg cell

Question 9

malignant cell in hodgkin dz originates from what type of WBC

Answer 9

B cell

Question 10

epidemiology of hodgkin dz

Answer 10

developed countries: bimodal age dist: young adulthood and after 50
more common in affluent families
developing countries - more common in young children

Question 11

clinical presentation of hodgkin dz

Answer 11

lymph node enlargement (cervical, mediastinal)
B sx common
Pel-Ebstein fever - relapsing high grade fever
pruritis
pain on drinking alcohol
immune deficiency

Question 12

how does hodgkin dz tend to spread?

Answer 12

contiguously

Question 13

dx of hodgkin

Answer 13

need to do excisional biopsy - FNA not adequate

Question 14

tx of hodgkin dz

Answer 14

XRT alone for favorable early stage dz
otherwise:
early stage - chemo + XRT
advanced - chemo +/- XRT at bulky sites

Question 15

chemo regimens used for hodgkin lymphoma

Answer 15

AVBD -current standard:
adriamycin
bleomycin
vinblastine
dacarbazine

MOPP - older regimen
BEACOPP - adv stage w/ risk factors

Question 16

late complications of hodgkin dz

Answer 16

high incidence of second malignancies
leukemia, lung cancer, breast cancer, mostly related to XRT sites
hypothyroidism after radiation of neck
constrictive pericarditis
infertility
heart failure

Question 17

Which of the following is usually not assoc w/ NHL at presentation?
Anemia
Fever
Leukopenia
Lymphadenopathy
Weight loss

Answer 17

leukopenia

Question 18

Which of the following stage 3 lymphomas are more likely to be curable w/ chemotherapy?
Diffuse large b cell lymphoma
Follicular
Marginal zone
Small lymphocytic

Answer 18

diffuse large b cell

Question 19

Which of the following lymphomas is expected to be CD4 pos?
Burkitt
Diffuse large b cell
Follicular
Hodgkin
Mycosis fungoides

Answer 19

Mycosis fungoides

Question 20

Following assoc btwn specific lymphoma and microorganism are correct except:
Adult t cell leuk/lymp & HTLV1
Burkitt and EBV
Hodgkin and parvo b19
Marginal zone and h pylori

Answer 20

Hodgkin and parvo b19 (acutally assoc w/ EBV)

Question 21

55 yo male w/ 2 mo hx of progressively inc L cervical LNs. Non smoker. FNA shows malignant B cells consistent w/ NHL. Next step?
Ct w/ contrast
Ct w/o contrast
Excisional biopsy
Chemo w/ R-CHOP
Chemo w/ ABVD

Answer 21

excisional biopsy

Question 22

Which therapy is less likely to be useful in tx of pt w/ biopsy proven, symptomatic stage 2 anaplastic large cell lymphoma?
Multi agent chemo
Radiation
Combined modality therapy (chemo + rad)
Surgical resection of affected lymph nodes

Answer 22

surgical resection

Question 23

22 yo male w/ 2 mo hx of fever, 15% wt loss, night sweats. Further eval reveals large mediastinal mass. Biopsy > hodgkin lymphoma. Which is least important in planning therapy?
Evidence of bone marrow invlvmt
Hx of b sx
Mixed cellularity histo
Presence of addl areas of lymphadenopathy
Size of mass

Answer 23

mixed cellular histo