Aggressive NHLs

Question 1

origin of diffuse large b cell lymphomas

Answer 1

evolve from low grade lymphomas or arise de novo

Question 2

cytogenetic hallmark of burkitt lymphoma

Answer 2

t(8;14)

Question 3

clinical features of DLBCL

Answer 3

present with rapidly enlarging symptomatic mass at a single nodal or extra nodal site. painful. stage 4 isn’t common.

Question 4

most common extra nodal site of DLBCLs

Answer 4

GI tract. bone marrow and peripheral blood involvement is rare

Question 5

morphology of DLBCLs

Answer 5

homogenous fish flesh tissue replacement (soft since lymphocytes aren’t meant to stick together), presence of hemorrhage, necrosis, fibrosis

Question 6

microscopic appearance of DLBCLs

Answer 6

diffuse architectural effacement, large cells, mitotically active, invasive, necrosis. can be centroblastic (oval vesicular nuclei, ugly cells) or immunoblastic (large nucleus, lots of cytoplasm)

Question 7

international prognostic index for DLBCLs

Answer 7

do less well if >60, have elevated LDH, poor fitness, extensive disease on both sides of diaphragm, multiple extra nodal sites

Question 8

GCB type of DLBCL

Answer 8

germinal center B cell type, express BCL6/10, tend to respond better to treatment

Question 9

ABC type of DLBCL

Answer 9

activated B cell type, have traversed the germinal center, don’t respond well to surgery

Question 10

three types of burkitt lymphoma

Answer 10

endemic, sporadic, immunodeficiency associated

Question 11

endemic BL

Answer 11

most common childhood malignancy in Africa, males more, always associated with EBV

Question 12

sporadic BL

Answer 12

affects young adults, males more. sometimes associated with EBV

Question 13

immunodeficiency associated BL

Answer 13

primarily seen in association with HIV. can be the initial manifestation of AIDS, sometimes with EBV

Question 14

definition of BL

Answer 14

aggressive extranodal lymphoma and/or leukemia with propensity for CNS involvement

Question 15

sites of involvement in endemic BL

Answer 15

facial bones, ileocecum, ovaries, kidneys, breast

Question 16

sites of involvement in sporadic BL

Answer 16

abdominal masses most common, ovaries, kidneys, breast

Question 17

sites of involvement in immunodeficiency related BL

Answer 17

lymph nodes and bone marrow

Question 18

clinical features of burkitt lymphoma

Answer 18

rapid doubling time, high tumor burden, bulky disease, tumor lysis syndrome

Question 19

BL prognosis

Answer 19

potentially curable but treatment needs to begin asap

Question 20

BL morphology

Answer 20

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)

Question 21

BL and Ki67

Answer 21

100% proliferative rate

Question 22

derivation of burkitt lymphoma

Answer 22

germinal center b cell

Question 23

immunophenotype of burkitt lymphoma

Answer 23

CD19/20+ (mature B cell)
CD5- (not t cell)
light chain restricted
CD10+, BCL+, BCL2- (not follicular!)

Question 24

cause of burkitt lymphoma

Answer 24

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)