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SADL Oncology > Lymphoma > Flashcards

Lymphoma Flashcards

1
Q

Immunophenotype of mature B-cell lymphomas

A

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

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2
Q

Burkitt’s translocations

A

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

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

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

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3
Q

Types of Burkitt’s lympoma

A

Endemic - 95%

EBV related Sporadic - 15%

EBV related Immunodeficiency-associated - 40% EBV

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4
Q

Grouping of Mature B NHL for treatment

A

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%

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5
Q

PTLD subtypes

A

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

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6
Q

Lymphoblastic lymphoma immunophenotype

A

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

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

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7
Q

Histology of Hodgkin lymphoma

A

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

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8
Q

Immunophenotype of classical vs nodular lymphocyte predominant Hodgkin’s

A

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

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9
Q

Key points of NLPHL

A

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

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10
Q

Key pathway in Hodgkin

A

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

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11
Q

Classic presentation of HL

A
  • 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)
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12
Q

Mediastinal mass differential

A

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

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13
Q

Role of PET in HL

A
  • staging - response assessment
  • NOT for surveillance post therapy.

Most relapse detected clinically < 12 m off therapy

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14
Q

Ann Arbor classification definition of extra letters and bulk

A

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

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15
Q

Prognostic factors

A
  • Stage
  • B symptoms
  • Bulk disease
  • Extra-nodal extension
  • Poor response to therapy
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16
Q

Most common secondary malignancy in HL

A

Breast cancer in women

  • highest for girls <10

Greatest relative risk is for leukemia

17
Q

Which tumors are CD 30 positive

A
  • cHodgkin
  • ALCL
  • PMBCL
  • embryonal carcinoma
18
Q

Late effects of HL therapy

A
19
Q

Which HL patients can avoid radiation?

A
  • 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
Q

What are the prognostic factors for relapsed HL?

A
  • B symptoms
  • Early relapse (3-12 months from end of therapy)
  • Failure to respond to second-line therapy
21
Q

What are the treatment options for relapsed HL?

A
  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
Q

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

A

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
Q

Treatment for low risk HL

A

2 cycels of OEPA

rads can be omitted unless poor response

24
Q

Treatment of intermediate risk HL

A

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
Q

Treatment for HR HL

A
  • 2 cycels OEPA then PET
  • 4 cycles COPDAC
  • Rads
26
Q

Diffuse Large B-cell Lymphoma

  • immunophenotype
  • cytogenetics
  • subtypes
A
  • 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
Q

ALCL

  • immunophenotype
  • translocations
A
  • immuno: CD15-CD30+ CD45+, Tcell marker: CD3, 43, TCR rearrangement
  • 75% have t(2;5) translocation with NPM-ALK
28
Q

Presentation of NHL

A
29
Q

St. Jude Classification

A

SADL Oncology (17 decks)

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