Non-Hodgkin's Lymphoma

Question 1

Adult types

Answer 1

Follicular lymphoma
CLL/SLL
Mantle cell

Question 2

Children and adolescent types

Answer 2

Burkitt
ALL/lymphoblastic
Anaplastic large T cell

Question 3

IPI for NHL

Answer 3

Age>60
Stage 3 or 4
Extranodal sites>2 
Performance status >2
LDH>normal

Question 4

What helps classify NHL?

Answer 4

Immunophenotype

Question 5

Small lymphocytic lymphoma severity and presentation

Answer 5

INdolent
Small B lympho and prolymphocytes
Advanced stage presentation
Older adults

Question 6

Small lymphocytic Lymphoma adverse

Answer 6

ZAP70+ (signlaing associated with Ig receptor)
p53 mutation
Naive B cell origin

Question 7

Richter syndrome

Answer 7

Transformation of small lymphocytic lymphoma to large B cell lymphoma

Question 8

Related leukemia to small lymphocytic lymphoma

Answer 8

CLL

Question 9

How can you distinguish SLL from mantle cell

Answer 9

Immuophenotype and lack of cyclin D1 overexpression in SLL

Question 10

Symptoms of SLL

Answer 10

Immune dysfunction with hypogammaglobulinemia, AIHA, and/or thrombocytopenia

Question 11

Follicular lymphoma severity and presentation

Answer 11

Indolent
Nodular proliferation of germinal center…mix of small and large cell
Generalized LAD
Usually high stage

Question 12

Follicular lymphoma mutation and how to ID

Answer 12

t(14;18) IGH/BCL2 overexpression (antiapoptotic protein)

Can use BCL2 stain…if reactive follicular hyperplasia then stains outside of germinal center

Question 13

Follicular lymphoma transformation

Answer 13

Transform to diffuse large B cell lymphoma with acquisition of additional abnormalities (from somatic hypermutation)

Question 14

Mantle Cell Lymphoma presentation and appearance

Answer 14

Looks indolent but aggressive

Adult
Usual nodal but may present as spleni of GI dz (lymphomatous polyposis)

Could be blastoid (look like lymphoblastic) or pleomorphic (looks like DLBCL)

May resemble FL, SLL, MZL

Question 15

Mutation in mantle cell lymphoma and how to ID

Answer 15

BCL1 (cyclin D1 overexpression)
t(11;14) IGH/cyclin D1

Use cyclin D1 immunohistochemical stain to diagnose

Question 16

Marginal zone B cell lymphoma of MALT appearance

Answer 16

Indolent 
Adult 
Small Bs, lymphoepithelial lesions and plasma cells 
Extranodal (GI most common)
Areas of ongoing inflammation

Question 17

Marginal B cell lymphoma of MALT association and mutation

Answer 17

Infectious agent (H.pylori) or autoimmunity

If t(11;18), then resistant to antimicrobial RX

Some mutations result in NFKB (pro-survival protein)

Question 18

DLBCL presentation and prognosis

Answer 18

Commonest in Western world
Aggressive B cell lymphoma of large lymphoid cells
Germinal center more favorable than activated type

Question 19

Mutation of DLBCL

Answer 19

BCL6 mutation prevents cell from maturing past germinal center stage
May also have BCL 2 mutation t(14;18)

Question 20

DLBCL associations

Answer 20

EBV, HHV-8

Question 21

Primary mediastinal DLBCL

Answer 21

Young females with SVC syndromes

Question 22

Primary CNS DLBCL

Answer 22

Immunosuppressed and elderly

Question 23

Burkitt’s lymphoma presentation

Answer 23

Agggressive mature B cell lymphoma 
Children and adults 
Africa or HIV 
Extranodal>nodal 
Ileocecal mass (sporadic) or jaw (endemic)

Question 24

Burkitt’s Lymphoma mutation, association and appearance

Answer 24

Ki67 proliferative index nearly 100% with starry sky
Basophilic cytoplasm
EBV
t(8;14) IGH/cMYC mutation…could also be t(2;8) or t(8;22)
Often have p53 mutation

Question 25

Peripheral T cell lymphoma

Answer 25

Very aggressive Adult Mature T cell phenotype Much less common

Question 26

Adult T cell leukemia/lymphoma

Answer 26

HTLV-1 associated lymphoma Japan, West Africa, Caribeean Skin lesions, hypercalcemia, LAD, HSM, flower cells Poor prognosis

Question 27

Extranodal NK/T cell lymphoma nasal type

Answer 27

``` Lethal midline granuloma Epistaxis, obstruction Ulcer/mass Vasculitis with malignant cytology EBV associated Poor prognosis ```

Question 28

Mycosis fungoides/Sezary Syndrome

Answer 28

T lymphoma of skin Cerebriform nuclei and paurtier microabscesses Indolent Erythroderma and bloodinvolvement

Question 29

T-lymphoblastic lymphoma

Answer 29

``` Very aggressive Normally children Mediastinal involvement Lymphoblasts Related to ALL B lymphoblastic is much less common ```

Question 30

Anaplastic large T-cell lymphoma, ALK+

Answer 30

Aggrssive but curable Young and male Extranodal Hallmark cells t(2;5) NPM/ALK (anaplastic lymphoma kinase) Unusual among T cell lymphomas because good prognosis

Question 31

BCL6, MYC, and BCL2 functions

Answer 31

6-maturation MYC - prolif BCL2- survive

Question 32

BCL2 mutation leads to

Answer 32

Follicular lymphoma

Question 33

Cyclin D1 mutation leads to

Answer 33

Mantle cell

Question 34

MYC mutation leads to

Answer 34

Burkitt, DLBCL

Question 35

FL mutation

Answer 35

t(14;18) - BCL2

Question 36

Mantle cell L mutation

Answer 36

t(11;14) - BCL1 (cyclin D1)

Question 37

CLL/SLL mutation

Answer 37

+12,13q,-17q ----p53

Question 38

MALT mutation

Answer 38

t(11;18), +3 ----MALT1

Question 39

DLBCL mutation

Answer 39

BCL6,BCL2, MYC

Question 40

BL mutation

Answer 40

t(8;14) ---- MYC

Question 41

ALCL mutation

Answer 41

t(2;5) ---- ALK

Question 42

Tx for most NHL

Answer 42

Rituximab

Question 43

HIV related lymphomas

Answer 43

``` DLBCL Burkitt PLasmablastic Primary effusion Hodgkin ```