Aggressive NHLs Flashcards
origin of diffuse large b cell lymphomas
evolve from low grade lymphomas or arise de novo
cytogenetic hallmark of burkitt lymphoma
t(8;14)
clinical features of DLBCL
present with rapidly enlarging symptomatic mass at a single nodal or extra nodal site. painful. stage 4 isn’t common.
most common extra nodal site of DLBCLs
GI tract. bone marrow and peripheral blood involvement is rare
morphology of DLBCLs
homogenous fish flesh tissue replacement (soft since lymphocytes aren’t meant to stick together), presence of hemorrhage, necrosis, fibrosis
microscopic appearance of DLBCLs
diffuse architectural effacement, large cells, mitotically active, invasive, necrosis. can be centroblastic (oval vesicular nuclei, ugly cells) or immunoblastic (large nucleus, lots of cytoplasm)
international prognostic index for DLBCLs
do less well if >60, have elevated LDH, poor fitness, extensive disease on both sides of diaphragm, multiple extra nodal sites
GCB type of DLBCL
germinal center B cell type, express BCL6/10, tend to respond better to treatment
ABC type of DLBCL
activated B cell type, have traversed the germinal center, don’t respond well to surgery
three types of burkitt lymphoma
endemic, sporadic, immunodeficiency associated
endemic BL
most common childhood malignancy in Africa, males more, always associated with EBV
sporadic BL
affects young adults, males more. sometimes associated with EBV
immunodeficiency associated BL
primarily seen in association with HIV. can be the initial manifestation of AIDS, sometimes with EBV
definition of BL
aggressive extranodal lymphoma and/or leukemia with propensity for CNS involvement
sites of involvement in endemic BL
facial bones, ileocecum, ovaries, kidneys, breast
sites of involvement in sporadic BL
abdominal masses most common, ovaries, kidneys, breast
sites of involvement in immunodeficiency related BL
lymph nodes and bone marrow
clinical features of burkitt lymphoma
rapid doubling time, high tumor burden, bulky disease, tumor lysis syndrome
BL prognosis
potentially curable but treatment needs to begin asap
BL morphology
monotonous infiltrate of medium sized cells. starry sky pattern with numerous tingible body macrophages, deeply basophilic cytoplasm with lipid vacuoles (red on oil red stain)
BL and Ki67
100% proliferative rate
derivation of burkitt lymphoma
germinal center b cell
immunophenotype of burkitt lymphoma
CD19/20+ (mature B cell)
CD5- (not t cell)
light chain restricted
CD10+, BCL+, BCL2- (not follicular!)
cause of burkitt lymphoma
tremendous proliferative drive due to EBV infection (those who are T cell immunodeficient can’t clear), which leads to genetic accident/translocation and activation of cMYC via t(8;14)
