Hodgkin Lymphoma Flashcards

1
Q

The average age at diagnosis of HL is _____ years

A

32

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2
Q

What are the characteristic features that differentiate HL from NHL? (3)

A
  1. HL spreads from one lymph node to the next lymph node (contiguous spread)
  2. Lymph nodes involved are easily accessible in HL
  3. Reed-Sternberg cells are present w/ a non-neoplastic background of lymphocytes and granulocytes.
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3
Q

_____% of the cells in HL are reactive lymphocytes and other white cells

A

90%

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4
Q

Reed-Sternberg cells are derived from ______________ cells

A

germinal center or postgerminal center B

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5
Q

Name the types of HL

A
  1. Nodular sclerosis
  2. Mixed cellularity
  3. Lymphocyte-rich
  4. Lymphocyte depletion
  5. Lymphocyte predominance
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6
Q

______________ subtype is not included in the classical form because it has a different kind of RS cell

A

Lymphocyte predominance

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7
Q

How do we know that RS cell is derived from a B cell?

A

RS cells show 2 things
V(D)J recombination
Somatic hypermutation

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8
Q

Activation of the transcription factor ________is a common event in classical HL

A

NF-κB

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9
Q

NF-κB may be activated either by _____ infection

A

EBV

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10
Q

EBV+ tumor cells express ____________, a protein encoded by EBV genome that upregulates NF-KB activity

A

latent membrane protein-1

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11
Q

Activation of NF-κB may occur in EBV-tumors as a result of acquired loss-of-function mutations in ___________, which are both negative regulators of NF-κB

A

IκB or A20

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12
Q

What is the whole concept of HL?

A

t is hypothesized that activation of NF-κB rescues “crippled” germinal center B cells that cannot express Igs from apoptosis, setting the stage for the acquisition of other unknown mutations that collaborate to produce Reed-Sternberg cells

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13
Q

EBV-infected B cells resembling Reed-Sternberg cells are found in the lymph nodes of individuals with ____________________

A

infectious mononucleosi

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14
Q

What are the cytokines and chemokines made by RS cells?

A
IL-5
IL-10
M-CSF
Eotaxin
Immunomodulatory factor galectin 1
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15
Q

Eosinophils and T cells express ligands that activate the _____ and ______ receptors found on Reed-Sternberg cells, producing signals that up-regulate NF-κB

A

CD30

CD40

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16
Q

_____________ cells are aneuploid and possess diverse clonal chromosomal aberrations

A

Reed-Sternberg

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17
Q

What are the characteristics of RS cells

A

45μm in diameter
Multiple nuclei or a single nucleus with multiple nuclear lobes
Large inclusion-like nucleoli about the size of a lymphocyte (5 to 7μm)

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18
Q

_________ variants contain a single nucleus with a large inclusion-like nucleolus

A

Mononuclear

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19
Q

Lacunar cells are seen in the __________ subtype

A

nodular sclerosis

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20
Q

How does a lacunar cell look like?

A

Delicate, folded, or multilobate nuclei Abundant pale cytoplasm that is often disrupted during the cutting of sections, leaving the nucleus sitting in an empty hole

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21
Q

Reed-Sternberg cells undergo a peculiar form of cell death in which the cells shrink and become pyknotic, a process described as _____________

A

mummification

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22
Q

What are the features of lymphohisticytic variant?

A

Polypoid nuclei
Inconspicuous nucleoli
Moderately abundant cytoplasm

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23
Q

The classical Reed-Sternberg cells are positive for _____, ____, _____, and negative for other B cell markers, T cell markers and CD45

A

PAX5
CD15
CD30

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24
Q

What is the microscopic picture of nodular sclerosis subtype of HL?

A

Lacunar cells present
Collagen deposition
Lymphocytes, histiocytes, macrophages, eosinophils common
Few RS cells

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25
What are the lymph nodes involved in nodular sclerosis subtype of HL?
lower cervical supraclavicular mediastinal
26
What is the male to female ratio in nodular sclerosis subtype of HL?
1:1
27
In 10% of patients, pain occurs in the lymph nodes when ever they take ________
alcohol
28
What is the prognosis of nodular sclerosis subtype of HL?
excellent (bcz low RS cells and high lymphocytes)
29
Nodular sclerosis is usually EBV _________ (positive/negative)
negative
30
Most patients of nodular sclerosis HL are __________ (young/old)
young
31
Nodular sclerosis is diagnosed ________
early
32
Key features of nodular sclerosis HL
1. Lacunar cells 2. Collagen deposition 3. Reactive white cells present 4. Common in young 5. Male to female ratio 1:1 6. Good prognosis 7. Hard nodules are found on examination
33
``` The lacunar cells are CD15 ___ (+/-) CD30 ___ (+/-) CD20 ___ (+/-) ```
CD15 + CD30 + CD20 -
34
Nodular sclerosis HL usually presents as stage ___ or ____
usually stage I or II disease
35
What are the microscopic features of Mixed cellularity otype of HL?
Diagnostic RS cells and mononuclear variants are plenty | Other cells - lymphocytes, eosinophils, plasma cells, macrophages
36
What is the immunophenotype of mixed cellularity type of HL?
CD15+ CD30+ CD20-
37
Reed-sternberg cells are usually ________ (infected/not infected) by EBV
infected (in 70% cases)
38
Mixed cellularity is more common in _________ (males/females)
males
39
Mixed cellularity HL is more associated with _________ (young/old)
old
40
Mixed cellularity type HL has biphasic incidence. What does this mean?
It peaks in young age and again in adults older than 55
41
People with Mixed cellularity type HL usually present in stage ___ or ____
III or IV
42
Mixed cellularity type HL is associated w/ symptoms like _________ and _________
night sweats and weight loss
43
Overall prognosis of Mixed cellularity type HL is _________
good
44
What are the microscopic features of Lymphocyte Rich type HL
Rich of lymphocytes (T cells) | RS cells and mononuclear variants
45
How do we differentiate between lymphocyte rich and lymphocyte predominance type?
Lymphocyte rich has RS cells and mononuclear variants whereas lymphocyte predominant type has popcorn cells Immunophenotype is also different
46
Compare the immunophenotype of lymphocyte rich and predominant HL.
Lymphocyte rich = 15+ and 30+ | Lymphocyte predominance = 20+, 15- and 30-
47
Lymphocyte rich type is associated with EBV in ____% of cases
40%
48
Lymphocyte rich type has a _______ prognosis
good
49
Lymphocyte rich HL occurs more commonly in ______ (males/females) and in ________ (young/old)
males | old
50
Lymphocyte Depletion type is associated with EBV in ____% of the cases
90%
51
Lymphocyte depletion type is associated with three factors. Name them
Older HIV + regargless of age Living in developing countries
52
What are the microscopic features of lymphocyte depletion type of HL?
Very less lymphocytes | Abundance of RS cells
53
What is the immunophenotype of lymphocyte depletion type of HL
CD15+ | CD30+
54
lymphocyte depletion type is associated with ________ stage
advanced stage
55
Prognosis of lymphocyte depletion HL is _______ (better/worse) than other subtypes
worse
56
What are the microscopic features of lymphocyte predominance type of HL
Lymphohistiocytic variant Nodular infiltrate of lymphocytes Macrophages
57
WHat is the immunophenotype of LP type of HL
CD20+ BCL6+ CD15- CD30-
58
In some cases, LP type of HL may convert into ___________
B cell lymphoma
59
Association of LP type of HL with EBV is ________ (common/uncommon)
uncommon
60
LP type of HL is more common in ________ (male/female) and _________ (young/old)
males | young (less than 35)
61
Which lymph nodes are commonly involved in LP type of HL?
Cervical and axillary
62
LP type of HL can reccur and/but its prognosis is _________ (good/bad)
good
63
What are the nodules made of in LP type of HL?
expanded B cells follicles reactive B cells dendritic cells
64
WHat are the clinical features of HL in advanced stages?
Fever Night sweats Weight loss Anergy
65
What is anergy?
Cutaneous immune unresponsiveness due to low cell mediated immunity. RS cells make some factors that decrease T1H cells response
66
What is the way of spreading of HL?
First node then spleen then liver and then marrow and others
67
Cure rate if stage I and II =? | Cure rate if stage IVA or IVB =?
90% | disease free survival at 5 years is 60-70%
68
What is the best treatment for HL so that no malignancy can develop?
Anti CD30 antibodies | Less genotoxic agents (other than radiotherapy and alkylationg agents)