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FCPath(SA)Haem - HaemOnc > Hodgkin Lymphoma > Flashcards

Hodgkin Lymphoma Flashcards

1
Q

Two types of HL

A

**I. CHL **(95% of HL, 2 peaks in age, EBV associated, IMPT: CD15+, CD30+, CD45-)
* Nodular Sclerosing (70%)
* Mixed cellularity
* Lymphocyte rich
* Lymphocyte depleted

II. Non-CHL (5% of HL, 30-50 yo males, LP cells, IMPT: CD15-, CD30- and CD45+)
* Nodular lymphocyte predoninant

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2
Q

Treatment of HL

A

PDL-1 Inhibitors:

  • Pembroluzumab
  • Nivolumab
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3
Q

What is true of all the four subtypes of

CHL?

A
  • derived from B cells
  • mononuclear Hodgkin cells and multinucleated RS cells present in all
    • reduced expression of B cell antigens
      • CD20, CD79a and PAX5
    • positive for: CD15 and CD30
  • variable EBV association (subtype specific)
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4
Q

What are the 4 subtypes of

CHL?

A
  • Nodular Sclerosis
  • Lymphocyte rich
  • Mixed cellularity
  • Lymphocyte depleted
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5
Q

What is the Syncytial variant of

Nodular Sclerosing CHL?

A
  • lacunar cells form cohesive nests in the centres of the nodules
  • necrosis may or may not be present
    • if prominent should consider this variant
  • may think this is Anaplastic large cell lymphoma
  • PAX5 positive
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6
Q

Features of NS CHL

A
  • Nodules
  • Mediastinum and bulky disease
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7
Q

What two subtypes of CHL are often

associated with EBV infection?

A
  • Mixed cellularity CHL
  • Lymphocyte depleted CHL

Note: NLPHL is only rarely associated with EBV infection (<5%)

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8
Q

Features of MC CHL

A
  • Diffuse
  • Peripheral LNs and spleen
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9
Q

Features of LR CHL

A
  • Nodular
  • Peripheral LNs
  • B-Sx = rare
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10
Q

Features of LD CHL

A
  • > HRS : Lymphocytes
  • Intra-abdominal LNs and Extranodal disease
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11
Q

DDx for HRS cells

6

A
  1. HL
  2. EBV+ DLBCL
  3. 1’ DLBCL of CNS
  4. 1’ mediastinal LBCL
  5. 1’ Effusion lymphoma
  6. TCHR LBCL
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12
Q

DDx for CD30+ lymphomas

HL, BCL(5), TCL(5)

A

I. HL

II. BCL
* Anaplastic variant of DLBCL
* Lymphomatoid granulomatosis
* PBL
* ALK+ LBCL
* 1’ effusion lymphoma

III. TCL
* ATCL
* Extranodal NK/TCL
* Enteropathy ass. TCL
* PTCL, NOS
* ALCL

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13
Q

What is the morphology of Classic

Reed-Sternberg Cells?

A
  • large with abundant and slightly basophilic cytoplasm
  • have at least 2 nuclear lobes and 2 nuclei
    • can have more than this
  • nuclei are large, rounded contours
  • prominent, irregular nuclear membrane
  • pale chromatin, eosinophlic nucleolus
    • perinuclear halo
    • 2 prominent nucleoli in 2 separate nuclei in one cell
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14
Q

What is the EBV expression of HS cells

of CHL ?

A
  • EBV infected cells express LMP1 and EBNA1
    • no expression of EBNA2
    • this is characteristic of type II EBV infection
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15
Q

Hodgkin lymphomas generally affect which

organs and what is a common feature of

most of them?

A
  • usually affect Lymph Nodes
  • variable mixture of associated, non-neoplastic inflammatory cells
  • sometimes associated with variable fibrosis (thick band like)
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16
Q

What are the 2 types of

Hodgkin’s Lymphomas?

A
  • Nodular lymphocyte predominant (NLPHL)
  • Classic Hodgkin Lymphoma
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17
Q

Which HL is the most common type and

what age groups is it often seen in?

A
  • Classic Hodgkin Lymphoma
    • 90% of all HL
  • Two peaks
    • 15-35 years old
    • late life
  • Has different subtypes that fall into these categories
18
Q

How does HL (both types, general) present

clinically?

A
  • peripheral lymphadenopathy
    • usually localized to 1-2 lymph nodes
    • generally cervical
    • NLPHL prefers peripheral lymph nodes
  • B symptoms
    • fever, drenching night sweats, weight loss
    • NLPHL does not present with B symptoms, in fact it is usually asymptomatic
19
Q

At what stage of B cell maturation are

Hodgkin/R-S cells derived from?

A
  • > 98% of cases are neoplastic B cells
  • derived from mature B cells of the germinal centre stage
  • clonal IG genes
20
Q

What are the 4 subtypes of

Classical Hodgkin’s Lymphoma?

A
  • Nodular Sclerosis Hodgkin Lymphoma
  • Lymphocyte rich CHL
  • Mixed Cellularity CHL
  • Lymphocyte depleted CHL
21
Q

What age group is preferentially affected

by Nodular Lymphocyte Predominant HL?

A
  • generally 4th-5th decade
  • also common in children
  • M > F
22
Q

CHL subtypes tend to have a male predominance, with the exception of which subtype?

A
  • Nodular sclerosing HL
    • slight female predominance
23
Q

What is the morphology of the Hodgkin cell

and the Mummified Hodgkin cell?

A
  • smudgy large nuclei (may be slightly eosinophilic)
  • can be anaplastic and confused with ALCL
24
Q

What is the immunophenotype of the HS

of CHL?

A
  • CD15 and CD30- membranous pattern with accentuation in the Golgi region
  • PAX5
    • weaker than reactive B cells (IMP)
  • IRF4/MUM1 - strongly positive
  • CD20
    • positive in 30% of cases
  • CD79a
    • not often expressed
  • CD45 and CD68 - negative
25
Q

What is the expression of OCT2 and BOB1

in CHL HS cells?

A
  • absence of the transcription factor (OCT2) and its cofactor (BOB1)

Note: EMA is also negative in most cases

26
Q

How can CHL lymphoma be mistaken for ALCL

and what IHC helps differentiate the two well?

A
  • if the CHL is rich in neopastic cells it can morphologically resemble ALCL
  • IHC
    • CHL = PAX5 (+)
      • EMA and ALK protein (-)
    • Detection of EBV (LMP1) favors CHL over ALCL as well
27
Q

What is one of the most difficult d/d

with CHL to differentiate?

A
  • DLBCL with anaplastic morphology and CD30 (+) cells
    • especially if it is within the mediastinum
28
Q

What is the IHC findings of

NLPHL?

A
  • very different from CHL, preserve B cell programming
  • Positive:
    • CD20, CD79a, PAX5
    • OCT2, BOB1
  • Negative:
    • CD30 (can have patchy, weak +)
    • CD15
    • EMA
29
Q

In what subset of patients would you

more likely see IgD expression by the LP

cells in NLPHL?

A
  • IgD expression is usually seen in the LP cells of young males
30
Q

What T lymphocytes are found predominantly

in the cellular milieu of HL?

A
  • T helper 2 cells
  • thought to be a result of the abnormal cytokine production of CHL cells
31
Q

What is the normal counterpart to the HRS

cells of CHL?

A
  • clonal and Germinal center B cell derived
  • based on IG studies

Note: LP cells are clonal expansions of GCB cells as well but they have the classic immunophenotype

32
Q

What is the EBV expression/infection

pattern in the various types of HL?

A
  • Highest:
    • MCCHL
    • LDCHL
  • Lowest:
    • NLPHL

Note: NSCHL shows an intermediate range of EBV positivity

IMP: HIV and in resource poor countries EBV + is almost 100%

33
Q

What medications are part of the

ABVD protocol to treat CHL?

A
  • Doxorubicin
  • Bleomycin
  • Vinblastine
  • Dacarbazine
34
Q

What medications are included in the

escalated BEACOPP treatment protocol?

A
  • Bleomycin
  • Etoposide
  • Doxorubicin
  • Cyclophosphamide
  • Vincristine
  • Procarbazine
  • Prednisone
35
Q

What is the current prognosis of CHL

with modern treatments?

A
  • >85% of cases of CHL are curable

Note: in advanced stage disease the International Prognostic Index score correlates with prognosis

36
Q

What is a new drug that has been approved

for treatment of relapsed/refractory CHL?

A
  • Brentuximab vedotin
  • CD30 directed antibody

Note: anti-PD1 antibodies have also been investigated and have promising results

37
Q

What has been shown with tumor infiltrating

CD68+, histiocytes in CHL?

A
  • a high content of macrophages correlated with reduced progression-free survival
  • also a high content is a feature of some aggressive forms of CHL like lymphocyte-depleted subtype
38
Q

Treatment approach in CHL

A

PET adapted regimens with ABVD/BEACOPP
New: BEACOP-Dac/BV-AVD/N-AVD

Ann Arbor:

I. Early (I/II)
* Prognosis (GHSC) = Mediastinal/Bulky, ESR, Estra-Nodal and Nr of LN)
* Favourable: 2x ABVD & RT or 4-6x ABVD
* Advance: 4x ABVD & RT or 4-6 ABVD

II. Late (III/IV)
* RATHL = 2x ABVD then PET (Deauville score)
* 1-3 (PET neg) = ABVD x6
* > 3 (PET pos) = BEACOPP x6 or escBEACOPP x4
* GHSG = 2x BEACOPP the PET
* PET neg = 2x esc BEACOPP
* PET pos = 4x esc BEACOPP

39
Q

PET Deauville Score?

A

Nodal Uptake:

  • 0 = No uptake
  • 1 = Uptake but < than heart
  • 2 = Uptake > than heart but < than liver
  • 3 = Uptake > than liver
  • 4 = +++ > than liver
40
Q

Treatment of NLPHL

A

Ann Arbor stage

I. Early:
* Surgery
* RT
* W&W

II. > 2 sites or Late:
* W&W
* R-CVP/R-ABVD
* R-CHOP
* R

41
Q
A

FCPath(SA)Haem - HaemOnc (19 decks)

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