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