Non-Hodgkin's Lymphoma Flashcards

1
Q

Adult types

A

Follicular lymphoma
CLL/SLL
Mantle cell

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2
Q

Children and adolescent types

A

Burkitt
ALL/lymphoblastic
Anaplastic large T cell

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3
Q

IPI for NHL

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

What helps classify NHL?

A

Immunophenotype

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5
Q

Small lymphocytic lymphoma severity and presentation

A

INdolent
Small B lympho and prolymphocytes
Advanced stage presentation
Older adults

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6
Q

Small lymphocytic Lymphoma adverse

A

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

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7
Q

Richter syndrome

A

Transformation of small lymphocytic lymphoma to large B cell lymphoma

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8
Q

Related leukemia to small lymphocytic lymphoma

A

CLL

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9
Q

How can you distinguish SLL from mantle cell

A

Immuophenotype and lack of cyclin D1 overexpression in SLL

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10
Q

Symptoms of SLL

A

Immune dysfunction with hypogammaglobulinemia, AIHA, and/or thrombocytopenia

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11
Q

Follicular lymphoma severity and presentation

A

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

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12
Q

Follicular lymphoma mutation and how to ID

A

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

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

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13
Q

Follicular lymphoma transformation

A

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

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14
Q

Mantle Cell Lymphoma presentation and appearance

A

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

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15
Q

Mutation in mantle cell lymphoma and how to ID

A

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

Use cyclin D1 immunohistochemical stain to diagnose

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16
Q

Marginal zone B cell lymphoma of MALT appearance

A
Indolent 
Adult 
Small Bs, lymphoepithelial lesions and plasma cells 
Extranodal (GI most common)
Areas of ongoing inflammation
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17
Q

Marginal B cell lymphoma of MALT association and mutation

A

Infectious agent (H.pylori) or autoimmunity

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

Some mutations result in NFKB (pro-survival protein)

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18
Q

DLBCL presentation and prognosis

A

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

19
Q

Mutation of DLBCL

A

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

20
Q

DLBCL associations

A

EBV, HHV-8

21
Q

Primary mediastinal DLBCL

A

Young females with SVC syndromes

22
Q

Primary CNS DLBCL

A

Immunosuppressed and elderly

23
Q

Burkitt’s lymphoma presentation

A
Agggressive mature B cell lymphoma 
Children and adults 
Africa or HIV 
Extranodal>nodal 
Ileocecal mass (sporadic) or jaw (endemic)
24
Q

Burkitt’s Lymphoma mutation, association and appearance

A

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

25
Q

Peripheral T cell lymphoma

A

Very aggressive
Adult
Mature T cell phenotype
Much less common

26
Q

Adult T cell leukemia/lymphoma

A

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

27
Q

Extranodal NK/T cell lymphoma nasal type

A
Lethal midline granuloma
Epistaxis, obstruction 
Ulcer/mass
Vasculitis with malignant cytology 
EBV associated
Poor prognosis
28
Q

Mycosis fungoides/Sezary Syndrome

A

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

29
Q

T-lymphoblastic lymphoma

A
Very aggressive
Normally children
Mediastinal involvement
Lymphoblasts 
Related to ALL
B lymphoblastic is much less common
30
Q

Anaplastic large T-cell lymphoma, ALK+

A

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

31
Q

BCL6, MYC, and BCL2 functions

A

6-maturation
MYC - prolif
BCL2- survive

32
Q

BCL2 mutation leads to

A

Follicular lymphoma

33
Q

Cyclin D1 mutation leads to

A

Mantle cell

34
Q

MYC mutation leads to

A

Burkitt, DLBCL

35
Q

FL mutation

A

t(14;18) - BCL2

36
Q

Mantle cell L mutation

A

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

37
Q

CLL/SLL mutation

A

+12,13q,-17q —-p53

38
Q

MALT mutation

A

t(11;18), +3 —-MALT1

39
Q

DLBCL mutation

A

BCL6,BCL2, MYC

40
Q

BL mutation

A

t(8;14) —- MYC

41
Q

ALCL mutation

A

t(2;5) —- ALK

42
Q

Tx for most NHL

A

Rituximab

43
Q

HIV related lymphomas

A
DLBCL
Burkitt
PLasmablastic
Primary effusion
Hodgkin