Lymphoid & Myeloid Neoplasms Flashcards
Follicular lymphoma
t(14;18), translocations involving BCL2
-overexpression of BCL2
IgH locus chr
Chr 14
BCL2 chr
Chr 18
follicular lymphoma morph
centrocytes & centroblast
BM involvement = paratrabecular lymphoid aggregates
BCL6 is normal, BCL2 is not normal
Follicular lymphoma clinical
most common indolent lymphoma of adults
painless, generalized LAD, extranodal site involvement, indolent NHL, anti-CD20 Ab, can transform to DLBCL
DLBCL
most common form of NHL/most common lymphoma of adults
Males, 60 yrs, can also occur in young adults and children
DLBCL path
dysregulaton of BCL6- needed for norm germinal center formation
-express CD19, CD20, CD10, BCL6, surface Ig
primary effusion lymphom
DLCBL subtype
pleural effusion in HIV or older pts
tumor cells infx with KSHV/HHV8
immunodeficiency-associated large B-cell lymphoma
DLBCL subtype
severe T cell immunodef
neoplastic B cells infx with EBV
DLBCL clinical
rapidly enlarging mass at a nodal or extranodal site
waldeyer ring involved- oropharyngeal tissue that includes tonsils & adenoids
No BM involvement
-aggressive tumors, rapidly fatal without tx
-anti-CD20 antibody therapy
Burkitt lymphoma translocation
MYC gene on chr 8 -> lead to incr MYC protein levels
- incr expressions of genes required for aerobic glycolysis= warburg effect
- translocation partner usually IgH locus t(8;14)
endemic Burkitt lymphoma
latently infx with EBV
-predilection for mandible, may involve abdominal visceral organs -> kidneys, ovaries, adrenal glands
-25% HIV-associated tumors also infx w/ EBV
15-20% sporadic cases- EBV infx
Burkitt lymphoma morph
tumor exhibits high mitotic index and contains numerous apoptotic cells & tumor cells have multiple small nucleoli
- starry sky pattern- phagocytes w/ abundant clear cytoplasm (numerous pale tingible body M0s)
- tumor cells in BM aspirate -> royal blue cytoplasm containing clear cytoplasmic vacuoles
Burkitt lymphoma immunophenotype
CD19, CD20, CD10, BCL2, IgM
Burkitt lymphoma- clinical
found mainly in children or young adults
30% of childhood NHLs in US
Tumors manifest at extranodal sites
-involvement of BM and PB is uncommon
-Sporadic= mass involving the ileocecum & peritoneum
very aggressive, responds well to chemo
ALL
most common type of cancer in kids
precursor B & T cels
CLL/SLL
most common leukemia of adults tumor of mature B cells manifests w/ BM or LN involvement indolent course, incr suspectibility to infx and autoimmune disorders -prolymphocytes