Non-Hodgkin Lymphoma

Question 1

Burkitt lymphoma immunophenotype

Answer 1

CD19, CD20, CD79a (B-lineage)
surface immunoglobulin, CD10
high proliferation index (Ki-67)

Question 2

Burkitts - most common translocation

Answer 2

> 90% have MYC t(8;14)

Question 3

most common NHL in children

Answer 3

burkitts (40%)

Question 4

risk factors for NHL

Answer 4

• EBV (burkitt)
• immunodeficiency / immunosuppression
• ? pesticides - unclear

Question 5

immunodeficiency syndromes at increased risk of NHL

Answer 5

• hyper IgM
• IgA deficiency
• CVID
• chediak-higashi
• wiskott-aldrich
• x-linked agammaglobulinemia
• SCID

Question 6

immunodeficiency from chromosome instability - increased risk of NHL

Answer 6

• bloom syndrome
• ataxia telangiectasia
• nijegan breakage syndrome

Question 7

what are the main subsets of DLBCL?

Answer 7

• 90% are germinal center b cell-lke (GCB DLBCL)

- 10% are activated b cell like (ABC DLBCL - assoc with t(14;18))

Question 8

immunophenotype of lymphoblastic lymphoma

Answer 8

TdT positive - marker of immaturity. Most are T cell (75%) but 25% b cell

Question 9

three lymphoblastic lymphoma genetic changes that impact prognosis (EFS)?

Answer 9

NOTCH wt, PTEN mutated, LOH 6q - all do worse (EFS < 50%)

Question 10

fusion characteristic of ALCL

Answer 10

NPM-ALK fusion t(2;5)

seen in 75%

Question 11

immunophenotype in ALCL

Answer 11

CD30+, CD45+
T cell markers seen in 60% (CD3, CD4, CD45Ro)
CD15-

Question 12

how to differentiate burkitt’s and DLBCL?

Answer 12

BCL-6 is NOT expressed in burkitts but is found in DLBCL