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SADL Oncology > Lymphoma > Flashcards

Flashcards in Lymphoma Deck (29):
1

Immunophenotype of mature B-cell lymphomas

Burkitt: CD 10, 19, 20, 22, sIg+ (90%IgM), c-myc translocations

DLBCL: CD 19, 20, 22, 79a, PAX-5, sIg+ light chain restricted 2/3 BCL-6, 1/3c-myc

PMBCL: CD 19, 20, 22, 79a, PAX-5, CD30, negative for surface Ig but cytoplasm+ Gain in 9p

2

Burkitt's translocations

t(8,14) - IgH (80%)

t(2,8) - Igkappa (15%)

t(8,22) - Iglambda (5%)

3

Types of Burkitt's lympoma

Endemic - 95%

EBV related Sporadic - 15%

EBV related Immunodeficiency-associated - 40% EBV

4

Grouping of Mature B NHL for treatment

FAB:

Grp A - stage 1 resected or abdominal stage 2 resected EFS 98%

Grp B - not A or C - EFS 90%

Grp C - Leukemia (>25% blasts), CNS disease, non-responder to Grp B therapy. EFS 79%

5

PTLD subtypes

Early lesion Polymorphic Monomophic (i.e. looks like NHL) *90% are mature B-cell

6

Lymphoblastic lymphoma immunophenotype

B cell: Tdt positive, CD19, CD79a, or CD22 positive. HLA-DR +

 

T-cell: cytoplasmic or membrane CD3, TdT+, HLA-DR and CD34 negative

7

Histology of Hodgkin lymphoma

Nodular sclerosing Mixed cellularity Lymphocyte rich Lymphocyte depleted Nodular-lymphocyte predominant

8

Immunophenotype of classical vs nodular lymphocyte predominant Hodgkin's

classical: CD 15+, CD 30+. CD 45- NLPHL: CD 15-, CD 30-, CD 45 +

9

Key points of NLPHL

5% of HL 10-20% in pre-pubertal 75% are males Localized No B symptoms not bulky EBV negative Surgery alone sufficient for Stage 1A 10% transform to DLBCL

10

Key pathway in Hodgkin

NFkappaB activation leading to increased BCL-2 (anti-apoptotic) - EBV activation of LMP1 - mutations in IkB (negative regulator of NFkB) - increased CD30 signalling

11

Classic presentation of HL

- painless cervical, supraclav LN - Mediastinal mass (2/3) - Constitutional symptoms (10% wt loss in 6m, night sweats, fever x 3d) - evidence of inflammation (ESR, CRP, anemia,ferritin) - Immune dysreg (AIHA, ITP, AIN, nephrotic syndrome)

12

Mediastinal mass differential

Malignant: - HL, - NHL: lymphoblastic, DLBCL, PMBCL, ALCL - GCT - Soft tissue sarcoma - mets Non-malignant: - thymus - infectious: mycobacterium, EBV, toxo, histo - lymphoproliferative d/o - PTGC

13

Role of PET in HL

- staging - response assessment

- NOT for surveillance post therapy.

Most relapse detected clinically < 12 m off therapy

14

Ann Arbor classification definition of extra letters and bulk

A - asymptomatic

B - B symptoms

E - extralymphatic organ

S - splenic involvement

X - bulky mediastinal disease

Bulk: >1/3 thoracic diameter on PA CXR, nodal aggregate > 6cm on longitudinal axis

15

Prognostic factors

  • Stage
  • B symptoms
  • Bulk disease
  • Extra-nodal extension
  • Poor response to therapy

16

Most common secondary malignancy in HL

Breast cancer in women 

- highest for girls <10

 

Greatest relative risk is for leukemia

17

Which tumors are CD 30 positive

  • cHodgkin
  • ALCL
  • PMBCL
  • embryonal carcinoma

18

Late effects of HL therapy

19

Which HL patients can avoid radiation?

  • Low risk (1A, 2A) with CR post 2 cycles OEPA
  • Intermediate risk getting ABVE-PC and rapid early responder on PET after 2 cycles, and CR after 4 cycles (AHOD0031)

20

What are the prognostic factors for relapsed HL?

  • B symptoms
  • Early relapse (3-12 months from end of therapy)
  • Failure to respond to second-line therapy

 

21

What are the treatment options for relapsed HL?

  1. Chemo + auto HSCT  *current SOC
    1. Myeloablative (BEAM or CBV most common), wide range of EFS/OS reported
  2. Chemo + allo HSCT
    1. Historically only used if failed auto or primary refractory
  3. Targeted therapy
    1. Brentuximab (against CD30)
    2. Ritux (if CD20 positive)
    3. PD-1 inhibitors - pembrolizumab, nivolumab - high response rate in multiply relapse/heavily treated adult patients

22

What is the Deaville 5-point scale for PET response?

1) No uptake.
2) Uptake ≤ mediastinal blood pool.
3) Uptake > mediastinal blood pool and ≤ normal liver.
4) Moderately increased uptake > normal liver.
5) Markedly increased uptake > normal liver.

PET positive if 3/4/5

23

Treatment for low risk HL

2 cycels of OEPA

rads can be omitted unless poor response

24

Treatment of intermediate risk HL

Based on AHOD0031

  • 2 cycles ABVE-PC then PET
  • If RER - 2 more cycles of ABVE-PC. Can avoid rads if PET negative and no bulk
  • If SER and PET negative - 2 cycles ABVE-PC and IFRT
  • If SER and PET positive - 2 cycles DECA then ABVE-PC

25

Treatment for HR HL

  • 2 cycels OEPA then PET
  • 4 cycles COPDAC 
  • Rads

26

Diffuse Large B-cell Lymphoma

- immunophenotype

- cytogenetics

- subtypes

- CD19, 20, 22, 79a, Pax-5, CD40

- 2/3 BCL-6, 1/3 have cMYC

- 90% germinal center B-cell like, 10% activated b-cell like t(14;18)

27

ALCL

- immunophenotype

- translocations

- immuno: CD15-CD30+ CD45+, Tcell marker: CD3, 43, TCR rearrangement

- 75% have t(2;5) translocation with NPM-ALK 

28

Presentation of NHL

29

St. Jude Classification