Hodgkin Lymphoma Flashcards

1
Q

Hodgkin Lymphoma

What are the two peaks (age range) when there is increased incidence of HL (5.5 per 100,000)?

A

25 to 30

75 to 80

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2
Q

Hodgkin Lymphoma

It is the most common malignancy diagnosed among patients 15 to 19 years olds.

TRUE or FALSE?

A

True

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3
Q

Hodgkin Lymphoma

This disease has an equal male-female distribution,

TRUE or FALSE?

A

False

Male predominance (1.2 : 1)

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4
Q

Hodgkin Lymphoma

What virus has been proposed to have a relationship with the development of HL?

(This is also proposed as related to the development of the MCHL subtype in children in developing countries)

A

EBV

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5
Q

Hodgkin Lymphoma

What are the usual clinical presentations of HL?

A
  • painless adenopathy
  • incidental mediastinal mass on routine chest radiograph
  • systemic symptoms (unexplained fevers, drenching night sweats, weight loss, generalized pruritus, fatigue, and alcohol-induced pain in tissues involved by HL)
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6
Q

Hodgkin Lymphoma

Majority of involved sites are contiguous.

TRUE or FALSE?

A

True

Sites of involvement are typically contiguous, although occasional skip areas
occur.

The theory of contiguity of spread and the development of treatment
programs with radiation that included treatment of uninvolved sites were
important conceptual advances in the treatment of HL in the latter half of the
20th century.

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7
Q

Hodgkin Lymphoma

Explain the pathophysiology of the classic finding of ivory vertebra on plain radiographs

A

they are blastic changes on the bones due to hematogenous seeding

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8
Q

Hodgkin Lymphoma

Enumerate some laboratory findings that are considered adverse prognostic factors especially in advanced disease.

A

anemia
lymphopenia
leukocytosis
hypoalbuminemia

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9
Q

Hodgkin Lymphoma

Define “bulky” mediastinal adenopathy
3 ways

A

> 1:3 ratio to the maximum intrathoracic diameter (near the level of the diaphragm) on standing PA radiograph

> 10 cm

ratio >0.34 at the level of T5-T6 (EORTC)

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10
Q

Hodgkin Lymphoma

Definition of an enlarged cervical lymph node on contrast-enhanced CT?

A

> 1.5 cm

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11
Q

Hodgkin Lymphoma

In the absence of confirmatory findings of HL involvement in the spleen, a spleen of this size is considered likely involved per the current staging system.

A

> 13-cm long

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12
Q

Hodgkin Lymphoma

FDG’s uptake pattern in bone defines whether bone or bone marrow is involved, obviating the need for a bone marrow biopsy.

TRUE or FALSE?

A

True

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13
Q

Hodgkin Lymphoma

Specify the Deauville score:

No uptake

A

1

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14
Q

Hodgkin Lymphoma

Specify the Deauville score:

New areas of uptake unlikely to be related to lymphoma

A

X

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15
Q

Hodgkin Lymphoma

Specify the Deauville score:

Uptake moderately higher than liver

A

4

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16
Q

Hodgkin Lymphoma

Specify the Deauville score:

Uptake markedly higher than liver

A

5

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17
Q

Hodgkin Lymphoma

Specify the Deauville score:

New lesions

A

5

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18
Q

Hodgkin Lymphoma

Specify the Deauville score:

Uptake > mediastinum but ≤ liver

A

3

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19
Q

Hodgkin Lymphoma

Specify the Deauville score:

Uptake ≤ mediastinum

A

2

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20
Q

Hodgkin Lymphoma

Specify the Ann Arbor Stage:

Involvement of a single lymph node region

A

stage I

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21
Q

Hodgkin Lymphoma

Specify the Ann Arbor Stage:

Involvement of a single lymph node region and a localized extralymphatic organ on the same side of the diaphragm.

A

stage IIE

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22
Q

Hodgkin Lymphoma

Specify the Ann Arbor Stage:

Involvement of a single lymph node region with and an a diffuse extralymphatic organ on the same side of the diaphragm.

A

stage IV

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23
Q

Hodgkin Lymphoma

Specify the Ann Arbor Stage:

No lymph node involvement
Diffuse involvement of an extralymphatic organ on the same side of the diaphragm.

A

stage IV

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24
Q

Hodgkin Lymphoma

Specify the Ann Arbor Stage:

Involvement of 2 or more lymph node regions on the same side of the diaphragm.

A

Stage II

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25
Q

Hodgkin Lymphoma

Specify the Ann Arbor Stage:

Involvement of two lymph node regions on both sides of the diaphragm

A

III

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26
Q

Hodgkin Lymphoma

This system used X to designate bulky disease starting in 1989

A

Cotswolds modification of the Ann Arbor

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27
Q

Hodgkin Lymphoma

This system recommended deleting X to designate bulky disease starting in 2014 and instead note the size of the largest node

A

Lugano modification of the Ann Arbor

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28
Q

Hodgkin Lymphoma

What are two major categories of HL as defined by the WHO?

A

classic and nodular lymphocyte–predominant HL (NLPHL)

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29
Q

Hodgkin Lymphoma

Describe the morphology R-S cell, the signature neoplastic cell of HL.

A
  • binucleate
  • prominent nucleolus in each nucleus
  • well-demarcated nuclear membrane
  • perinuclear halo
  • eosinophilic cytoplasm
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30
Q

Hodgkin Lymphoma

Describe the immunohistochemical staining results of R-S cell, the signature neoplastic cell of HL.

A

Positive for:

CD30/lymphocyte activation marker

PAX5 (dimly)

CD20 (variably)

CD15/antigranulocyte monoclonal antibody (variably)

express PD-1/PDL-1 ligand

Negative for:
CD45
ALK
J Chain

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31
Q

Hodgkin Lymphoma

Enumerate the 4 subtypes of HL.

A

NSHL
MCHL
LRHL
LDHL

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32
Q

Hodgkin Lymphoma

This is characterized by lymph nodes that are diffusely effaced by lymphocytes, eosinophils, plasma cells,
inflammatory cells, and relatively abundant atypical mononuclear and R-S cells.

A

MCHL

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33
Q

Hodgkin Lymphoma

What is the most common histologic subtype diagnosed in developed countries?

A

NSHL

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34
Q

Hodgkin Lymphoma

It is characterized by involved nodes that often have a thick capsule and are traversed by
broad bands of birefringent collagen that surround nodules of cells consisting of
lymphocytes, eosinophils, plasma cells, and tissue histiocytes intermixed with a
variable proportion of atypical mononuclear cells, inflammatory cells, and R-S
cells

A

NSHL

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35
Q

Hodgkin Lymphoma

Identify the classical HL subtype:

Patients are usually young women, with a favorable natural history and typically present in the clinic with mediastinal involvement, and one-third have B symptoms.

A

NSHL

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36
Q

Hodgkin Lymphoma

Identify the classical HL subtype:

Patients usually present with advanced disease and tend to be slightly older and have a less favorable natural history than those with NSHL.

A

MCHL

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37
Q

Hodgkin Lymphoma

Identify the classical HL subtype:

It is difficult to differentiate this subtype from anaplastic large cell lymphoma especially because both are CD30+.

A

LDHL

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38
Q

Hodgkin Lymphoma

Identify the classical HL subtype:

It is the most uncommon, and has the worst prognosis of the four subtypes.

A

LDHL

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39
Q

Hodgkin Lymphoma

Identify the classical HL subtype:

This is confused with LPHL due to the clinical characteristics: early stage, absence of B symptoms, and excellent prognosis.

However, the staining characteristic is that of classical HL.

A

LRHL

40
Q

Hodgkin Lymphoma

Describe the staining characteristics of the L&H cells/popcorn cells of the NLPHL.

A
strongly positive for:
CD20
CD45
CD79a
PAX5

negative for:
CD15
CD30

41
Q

Hodgkin Lymphoma

What is the most common presenting profile of patients with NLPHL?

A

young people who present with early-stage disease, usually in a solitary
peripheral nodal site.

Systemic symptoms are uncommon

42
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Serum albumin

A

<4 g/dL

43
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Hemoglobin

A

<10.5 g/dL

44
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Gender

A

Male

45
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Age

A

≥45 y

46
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Ann Arbor Stage

A

IV

47
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Leukocyte count

A

> 15,000/mm3

48
Q

Hodgkin Lymphoma

Identify the values that would make a patient with advanced HL to have “unfavorable” prognosis based on the International Prognostic Scoring System

Lymphocyte count

A

<8% of WBC

or

<600/mm3

49
Q

Hodgkin Lymphoma

Chemotherapy.

What agents are included in ABVD?

A

doxorubicin
bleomycin
vinblastine
dacarbazine

50
Q

Hodgkin Lymphoma

Chemotherapy.

What agents are included in BEACOPP?

A
bleomycin
etoposide
doxorubicin
cyclophosphamide
vincristine
prednisone
procarbazine
51
Q

Hodgkin Lymphoma

Chemotherapy.

What agents are included in Stanford V

A
bleomycin
doxorubicin
vinblastine
vincristine
etoposide
prednisone
nitrogen mustard

(BEAVOPe) + nitrogen mustard

52
Q

Hodgkin Lymphoma

Targeted therapy.

This is an anti CD30 linked to an antitubulin which has demonstrated efficacy in CD30+ lymphomas.

This has also been approved as a maintenance therapy following autologous HCT (ETHERA trial).

A

BV

brentuximab vedotin

53
Q

Hodgkin Lymphoma

Targeted therapy.

This is checkpoint inhibitor approved for patients with disease that is chemoresistant or has relapsed after 3 or more lines of therapy.

A

Pembrolizumab

54
Q

Hodgkin Lymphoma

Targeted therapy.

This checkpoint inhibitor has been approved or patients whose disease has relapsed or progressed after autologous HCT and posttransplant brentuximab

A

Nivolumab

55
Q

Hodgkin Lymphoma

What is the ISRT treatment field recommended for “early” stage “favorable” disease that was treated with “abbreviated” chemotherapy?

A

ISRT

all initial sites of disease after completing chemotherapy

56
Q

Hodgkin Lymphoma

What is the ISRT treatment field recommended for NLPHL disease that will be treated with RT alone?

A

ISRT

CTV all initial sites + potenital subclinical disease (2- to 5-m margin within the lymphatic stations superior and inferior to the initially involved disease)

57
Q

Hodgkin Lymphoma

What is the ISRT treatment field recommended for “early” stage “unfavorable” disease that was treated with “abbreviated” chemotherapy?

A

ISRT to all sites of disease

58
Q

Hodgkin Lymphoma

What is the ISRT treatment field recommended for “early” stage “unfavorable” disease that was treated with “intensive” chemotherapy?

A

ISRT

certain complete responding areas that could result in unacceptable OAR doses can be omitted

59
Q

Hodgkin Lymphoma

What is the ISRT treatment field recommended for “advanced” stage disease?

A

ISRT to sites of initially bulky disease or sites of incomplete response

it is presumed that the patient was treated with intensive chemotherapy because of the advanced stage.

60
Q

Hodgkin Lymphoma

For patients with initially large mediastinal masses, what is the postchemotherapy CTV?

A

the width should conform to the residual disease only,

the superior and inferior border should encompass the initial extent of disease.

61
Q

Hodgkin Lymphoma

What is the NCCN recommended dose for RT alone?

A

30 to 36 Gy to involved sites
25 to 30 Gy to potential subclinical involvement

1.5- to 2.0-Gy per fraction

62
Q

Hodgkin Lymphoma

What is the NCCN recommended RT dose for RT as a component of combined modality treatment?

A

30 to 36 Gy to involved sites
1.5- to 2.0-Gy per fraction

Deauville 4-5 lesions may be treated from 36 to 45 Gy.

63
Q

Hodgkin Lymphoma

OARs

What are the doses at which there is an increased risk for RTOG grade 2 pneumonitis?

A

mean lung dose >14 Gy

V20 >35%

64
Q

Hodgkin Lymphoma

OARs

What are the threshold doses for RTOG grades 1-3 pneumonitis?

(doses should be keep below this)

A

mean lung dose 13.5 Gy

V20 <30

V5 <55

65
Q

Hodgkin Lymphoma

OARs

The risk of radiation-related pneumonitis s also increased with the use of what chemotherapy or targeted agent/s that also cause significant pulmonary toxicities.

A

bleomycin and BV

(they should be never used simultaneously)

nivolumab and pembrolizumab as well.

66
Q

Hodgkin Lymphoma

OARs

What is the threshold dose for the development of secondary lung cancer?

A

5 Gy

67
Q

Hodgkin Lymphoma

OARs

What is the threshold dose for the development of secondary breast cancer?

A

4 Gy

68
Q

Hodgkin Lymphoma

OARs

What is the relationship of coronary heart disease and mean heart dose?

A

the excess relative risk
(ERR) was 7.4% per Gy of MHD.

The ERR was negligible with an MHD <5
Gy and was approximately 2 for an MHD of 12 Gy

69
Q

Hodgkin Lymphoma

OARs

At what dose is there an increased ERR for valvular diseases?

A

> 20 Gy

70
Q

Hodgkin Lymphoma

OARs

At what dose is there an increased risk for congesitve heart failure?

A

left ventricular mean dose exceeds 15 Gy, or V30 is ≥50%

71
Q

Hodgkin Lymphoma

OARs

At what dose to the pericardium is there an increased risk for pericarditis?

A

> 30 Gy esp if using mantle field

72
Q

Hodgkin Lymphoma

Unfavorable disease characteristics of early stage I to II HL

EORTC

A

age ≥50

ESR ≥30 with any B sx
ESR ≥50 without B sx

Mediastinal mass MTR ≥0.35 (T5-6 level)

> 3 nodal regions

73
Q

Hodgkin Lymphoma

Unfavorable disease characteristics of early stage I to II HL

GHSG

A

ESR (similar to EORTC)
ESR ≥30 with any B sx
ESR ≥50 without B sx

MMR > 0.33

> 2 nodal regions

Any extralymphatic involvement

74
Q

Hodgkin Lymphoma

Unfavorable disease characteristics of early stage I to II HL

NCIC

A

Age ≥40
MC/LD histology

ESR ≥50 OR any B sx

MMR > 0.33

> 3 nodal regions

75
Q

Hodgkin Lymphoma

Unfavorable disease characteristics of early stage I to II HL

NCCN

A

Any B sx

ESR >50 mm

10 cm mass or MMR > 0.33

> 3 nodal regions

76
Q

Hodgkin Lymphoma

What is the treatment for early stage favorable HL with only 1 or 2 disease sites, <50 EST, no extranodal disease?

A

2 cycles of ABVD + 20 Gy ISRT

GHSG

77
Q

Hodgkin Lymphoma

What is the standard treatment for early stage favorable HL?

No interim PET was done.

A

CMT

4 cycles of ABVD + 30 Gy ISRT

78
Q

Hodgkin Lymphoma

What is the standard treatment for early stage favorable HL?

Consider a negative interim PET CT (Deauville 1 or 2).

A

CMT

3 cycles of ABVD (or 8 weeks of Stanford V) + 30 Gy ISRT

79
Q

Hodgkin Lymphoma

What is the standard treatment for early stage favorable HL?

Consider a positive interim PET CT after 2 cycles of ABVD but no progressive disease

A

additional 2 cycles of ABVD + 30 Gy ISRT

or 2 cycles of escalated BEACOPP + 30 Gy ISRT.

80
Q

Hodgkin Lymphoma

Can early-stage favorable HL be treated with ABVD alone?

A

yes.

(NCIC-CTG HD.6 trial).

12 years 94% survival rate
87% PFS.

81
Q

Hodgkin Lymphoma

What is the CTV expansion for ISRT in NLPHL (Stage I to IIA)

A

The CTV expansion must account for possible
microscopic spread beyond the lymph nodes clinically observed on PET-CT
imaging.

For a patient who presents with unilateral level I or IIA
disease, the CTV should be expanded to include ipsilateral levels IIB, III, and
even IV. For a femoral node presentation, the CTV should include at least a 5-cm
extension along the lymphatic chains superiorly and a 2-cm extension
inferiorly

82
Q

Hodgkin Lymphoma

Based on ESMO and NCCN guidelines,

what are the treatment recommendations for stage I and limited stage II respectively?

A

RT alone for stage I

CMT for stage II, unless it is non bulky with no more than 2 contiguous sites involved.

83
Q

Hodgkin Lymphoma

What is the standard treatment most commonly used for stage I or II HL with large mediastinal adenopathy?

A

4-6 ABVD + 30 Gy ISRT.

(However, EORTC H9U showed no significant benefit to 6 cycles vs 4 when given with RT).

84
Q

Hodgkin Lymphoma

What is the IFRT dose if BEACOPP is used for patients with intermediate prognosis?

A

20 Gy IFRT.

However, BEACOPP is more toxic than ABVD hence, 4 ABVD + 30 Gy IFRT can be considered.

85
Q

Hodgkin Lymphoma

Can you omit RT for stage III to IV HL?
When?

A

Yes.
If there is CR to a full course of conventional chemotherapy.

However, RT is often added for patients with bulky disease or suspected residual sites.

86
Q

Hodgkin Lymphoma

What is the dose for RT given prior to HCT?

A

similar to TBI (12 to 15 Gy)

87
Q

Hodgkin Lymphoma

If the RT will be given after HCT, when do you do it and what are your targets?

A

1 to 4 months after.

to sites of bulky disease at the time of relapse or sites that remain positive on PET before transplant.

You may also include all sites involved at the time of relapse.

88
Q

Hodgkin Lymphoma

What is the dose for RT given after HCT?

A

18 to 40 Gy

In general, lower
doses were employed when initially nonbulky disease was included in the
treatment or when there was a CR to high-dose therapy.

89
Q

Hodgkin Lymphoma

Follow-up schedule during the first 2 years following treatment.

A

every 3 to 6 months

90
Q

Hodgkin Lymphoma

Follow-up schedule after 2 years following treatment.

A

every 6 to 12 months

91
Q

Hodgkin Lymphoma

Identify:

This develops in approximately 10% to 15% of patients after radiation therapy to a significant length of the spinal cord via AP/PA fields.

It is more likely to occur among patients who have been treated with vinca alkaloids (vincristine and vinblastine).

A

Lhermitte sign

92
Q

Hodgkin Lymphoma

An uncommon but potentially serious complication is overwhelming sepsis after splenectomy or splenic irradiation.

What are the most common organisms involved?

A

Streptococcus pneumoniae

Hemophilus influenzae type b

Neisseria meningitidis

93
Q

Hodgkin Lymphoma

What chemotherapy regimen is considered safe to avoid male infertility following HL treatment?

A

ABVD and Stanford V

MOPP and BEACOPP can cause sterility

94
Q

Hodgkin Lymphoma

What chemotherapy regimen is considered safe to avoid female infertility following HL treatment?

A

ABVD and Stanford V

MOPP and BEACOPP can cause sterility

95
Q

Hodgkin Lymphoma

What secondary lymphoma is most common after treatment of HL?

A

DLBC

96
Q

Hodgkin Lymphoma

What disease has a potential risk of developing if a large portion of the pericardium is treated, which is uncommon in the current management programs; it presents as an acute febrile syndrome associated with chest pain and friction rub, an asymptomatic pericardial effusion diagnosed by chest radiograph or echocardiogram, or constrictive pericarditis or tamponade. Mild manifestations can be managed with conservative medical treatment including analgesics and nonsteroidal antiinflammatory agents; it usually clears within a few weeks.

A

Radiation pericarditis