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WHO Chapter 8: HL

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Hodgkin Lymphoma
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Page 1: WHO Chapter 8: HL

Hodgkin Lymphoma

Page 2: WHO Chapter 8: HL

Hodgkin Lymphoma

30% of all lymphomas

Absolute incidence unchanged

Arise in lymph node, cervical region

Neoplastic tissues usually contain a small

number of tumor cells

Page 3: WHO Chapter 8: HL

Incidence

Bimodal age incidence 15-40, >55 years

Childhood form (0-14) more common in

developing countries

M:F=1.5:1; in all subtypes except NS

Page 4: WHO Chapter 8: HL

W.H.O Classification

Nodular lymphocyte predominant Hodgkin

Lymphoma (NLPHL)

Classical Hodgkin lymphoma

Nodular sclerosis (NSHL)

Mixed cellularity (MCHL)

Lymphocyte rich (LRHL)

Lymphocyte-depleted (LDHL)

Page 5: WHO Chapter 8: HL

Neoplastic cells and Hodgkin

Lymphoma

Classical Hodgkin (HRS cells):

Reed-Sternberg cell

Hodgkin cell (mononuclear)

Lacunar cell

Nodular LP Hodgkin:

LP (lymphocyte predominant) cells, also known as

L&H (lymphocytic and histiocytic) cells, “Popcorn”

cells

Page 6: WHO Chapter 8: HL

NLPHL

5% of Hodgkin lymphoma

Male; mid 30’s

Bimodal age distribution not seen

Most present with localized peripheral

lymphadenopathy, develops slowly and is

responsive to therapy

Page 7: WHO Chapter 8: HL

NLPHL

Tends to spare mediastinum, spleen or BM

Association with or progression to DLBCL (2-

3%)

Analogous to “low grade” B-cell lymphomas;

but: (1) disseminated disease not usually seen,

and (2) younger age.

EBV negative

Page 8: WHO Chapter 8: HL

NLPHL

Architecture:

Nodular

Nodular and diffuse

Page 9: WHO Chapter 8: HL

NLPHL

Page 10: WHO Chapter 8: HL

LP (L&H) cells

Page 11: WHO Chapter 8: HL

LP (L&H)

cells

Page 12: WHO Chapter 8: HL

Immunophenotype

(LP cells)

CD45+

CD20+

EMA+ in 50% of cases

CD 15 and CD30 are negative

Page 13: WHO Chapter 8: HL

CD 20

LP cells and the back ground cells are CD20

positive; CD20 can be used to highlight the

nodularity

Page 14: WHO Chapter 8: HL

CD20 and NLPHL

Page 15: WHO Chapter 8: HL

EMA

Page 16: WHO Chapter 8: HL

CD30

Page 17: WHO Chapter 8: HL

CD57

CD57 (+) T cells surround LP cells

Page 18: WHO Chapter 8: HL

PTGC

(Progressively transformed germinal centers )

Page 19: WHO Chapter 8: HL

PTGC

Page 20: WHO Chapter 8: HL

PTGC

Progressively transformed germinal centers

(PTGC) are seen in association with NLPHL.

It is uncertain whether these lesions are

preneoplastic

Most patient with reactive hyperplasia and

PTGC do not develop HL

Page 21: WHO Chapter 8: HL

NLPHL

Prognosis is good especially for earlier stage

2-3% of cases progress to large B-cell

lymphoma

Page 22: WHO Chapter 8: HL

Classical Hodgkin lymphoma

95% of Hodgkin lymphomas

Bimodal age distribution

EBV has been postulated to play a role (lack of

immune surveillance)

Page 23: WHO Chapter 8: HL

Sites of involvement

Cervical lymph nodes

60% have mediastinal invlovement

Bone marrow involvement rare (5%) – stage IV

disease

Page 24: WHO Chapter 8: HL

Hodgkin Lymphoma

Fish-flesh tumor

Page 25: WHO Chapter 8: HL

Hodgkin Lymphoma Malignant Cell Variants

Mononuclear Hodgkin Cell Lacunar cells seen in nodular

sclerosis Hodgkin lymphoma

Page 26: WHO Chapter 8: HL

Hodgkin Lymphoma

Diagnostic Reed-Sternberg cell

Page 27: WHO Chapter 8: HL

Reed-Sternberg cell

Page 28: WHO Chapter 8: HL

RS cells

Page 29: WHO Chapter 8: HL

Mummified RS cell

Page 30: WHO Chapter 8: HL

Defining characteristics

RS cells in the appropriate cellular background

Page 31: WHO Chapter 8: HL

Immunophenotype

(HRS cells)

CD45- , CD15+ , CD30+, PAX5+

The neoplastic cells are usually not CD20 positive

The background lymphocytes are T cells (CD3

positive, CD20 negative)

Page 32: WHO Chapter 8: HL

RS cells and CD15

Page 33: WHO Chapter 8: HL

CD15

RS Cell

RS Cell

Neutrophil

Neutrophil

Page 34: WHO Chapter 8: HL

CD30

Page 35: WHO Chapter 8: HL

PAX5

Page 36: WHO Chapter 8: HL

EBV

The prevalence of EBV in RS cells varies

according to the histological subtype:

Highest in mixed cellularity (75%)

Lowest in nodular sclerosis (10-40%)

Page 37: WHO Chapter 8: HL

EBV

EBV-encoded Latent

Membrane Protein 1 (LMP 1)

EBER-Insitu Hybridization

Page 38: WHO Chapter 8: HL

Hodgkin Lymphoma Nodular Sclerosis Type

Lacunar cell

RS cell

Page 39: WHO Chapter 8: HL

Nodular Sclerosis

Most common type

The only type of HL without a male

predominance

Page 40: WHO Chapter 8: HL

Nodular

Sclerosis

Page 41: WHO Chapter 8: HL

RS and lacunar cells

Page 42: WHO Chapter 8: HL

Nodular Sclerosis

Cellular phase

Fibrotic phase

Syncitial variant: an extreme form of the cellular

phase (prominent aggregates of HRS cells)

Page 43: WHO Chapter 8: HL

Mixed cellularity HL

(MCHL)

More frequent in patients with HIV infection

and in developing countries

A bimodal age distribution is not seen

Often showing granulomas

Page 44: WHO Chapter 8: HL

Mixed cellularity

Page 45: WHO Chapter 8: HL

Mixed cellularity

Page 46: WHO Chapter 8: HL

Lymphocyte rich classical

Hodgkin lymphoma

Nodular (common): background cells are B cells

Diffuse: background cells are T cell

Notes: -Hisology of Nodular type resembles NLPHL

-Histology of Diffuse type resembles TCR-HR LBCL

-No difficulty in diagnosis with HRS cell immunostains

Page 47: WHO Chapter 8: HL

(Nodular) lymphocyte rich HL

Page 48: WHO Chapter 8: HL

LRHD and CD20

Page 49: WHO Chapter 8: HL

CD30 and LRHL

Page 50: WHO Chapter 8: HL

CD57 and LRHL

(No resetting around HRS cells)

Page 51: WHO Chapter 8: HL

Lymphocyte depleted HL

Relatively depleted non-neoplastic lymphocytes

Rare subtype (<1% of cHL)

Median age 30-37

Often a/w HIV

More advanced stages and with B symptoms

compared to other subtypes

May have a sarcomatous pattern

May mimick ALTCL

Page 52: WHO Chapter 8: HL

Lymphocyte depleted HL

Page 53: WHO Chapter 8: HL

Lymphocyte depleted, diffuse

fibrosis

Page 54: WHO Chapter 8: HL

Classical Hodgkin : Prognosis

Prognosis is now based on the clinical stage

rather than the histological subtype.

Massive mediastinal disease is a poor prognostic

factor in NS type

Page 55: WHO Chapter 8: HL

Addendum:

Differential diagnosis

Non-Hodgkin lymphoma

LDHL, ALTCL, and T-cell rich/histiocyte rich

DLBCL may look histologically similar

Page 56: WHO Chapter 8: HL

ALTCL

Large/multinucleated cells with abundant

cytoplasm

CD20 (–)

T- markers positive, can be “null” phenotype

CD30 positive

ALK1 positive (except for the provisional

ALTCL-Alk1 neg)

Page 57: WHO Chapter 8: HL

ALCL

Page 58: WHO Chapter 8: HL

ALCL

Intra-sinusoidal infiltration

Page 59: WHO Chapter 8: HL

CD30

Page 60: WHO Chapter 8: HL

ALTCL

Two subtypes:

Systemic : ALK1 +, EMA +, Clusterin +

Primary cutaneous : ALK1 -, EMA –, Clusterin -

Page 61: WHO Chapter 8: HL

DLBCL, anaplastic variant

Page 62: WHO Chapter 8: HL

DLBCL, immunoblastic

Page 63: WHO Chapter 8: HL

DLBCL: CD20+

Page 64: WHO Chapter 8: HL

DLBCL: CD30(-)

Page 65: WHO Chapter 8: HL

Immunoprofile

NLPHL

CD45+ CD20+ CD15-

CD30- EMA+ PAX5+

CHL

CD45- CD3- CD20-

CD15+ CD30+ PAX5+

ALTCL

CD45+ CD20- CD3- CD4+

CD30+ ALK1+ EMA+

PAX5-

DLBCL

CD45+ CD20+ CD3-

CD30+/- PAX5+


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