16 - Lymphoproliferative Disorders 2 Flashcards
mycosis fungoides
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
leukemic form of mycosis fungoides
Sezary syndrome
anaplastic large cell lymphoma
aggressive T cell NHL
CD30 pos
skin often involved
translocation associated with good prognosis anaplastic large cell lymphoma
t(2;5) - express of ALK (anaplastic lymphoma kinase)
lymphoblastic lymphoma
highly aggressive T cell NHL
male adolescents
mediastinal mass
basically lymphoma form of ALL
adult T cell leukemia/lymphoma
highly aggressive assoc w/ HTLV1 mostly seen in Japan/Caribbean frequent hypercalcemia CD4 cells
prognosis for hodgkin dz
highly treatable and curable, even when disseminated
key cell of hodgkin dz
reed sternberg cell
malignant cell in hodgkin dz originates from what type of WBC
B cell
epidemiology of hodgkin dz
developed countries: bimodal age dist: young adulthood and after 50
more common in affluent families
developing countries - more common in young children
clinical presentation of hodgkin dz
lymph node enlargement (cervical, mediastinal)
B sx common
Pel-Ebstein fever - relapsing high grade fever
pruritis
pain on drinking alcohol
immune deficiency
how does hodgkin dz tend to spread?
contiguously
dx of hodgkin
need to do excisional biopsy - FNA not adequate
tx of hodgkin dz
XRT alone for favorable early stage dz
otherwise:
early stage - chemo + XRT
advanced - chemo +/- XRT at bulky sites
chemo regimens used for hodgkin lymphoma
AVBD -current standard: adriamycin bleomycin vinblastine dacarbazine
MOPP - older regimen
BEACOPP - adv stage w/ risk factors
late complications of hodgkin dz
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
Which of the following is usually not assoc w/ NHL at presentation? Anemia Fever Leukopenia Lymphadenopathy Weight loss
leukopenia
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
diffuse large b cell
Which of the following lymphomas is expected to be CD4 pos? Burkitt Diffuse large b cell Follicular Hodgkin Mycosis fungoides
Mycosis fungoides
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
Hodgkin and parvo b19 (acutally assoc w/ EBV)
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
excisional biopsy
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
surgical resection
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
mixed cellular histo