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
Q

What are the lymph nodes involved in nodular sclerosis subtype of HL?

A

lower cervical
supraclavicular
mediastinal

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26
Q

What is the male to female ratio in nodular sclerosis subtype of HL?

A

1:1

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27
Q

In 10% of patients, pain occurs in the lymph nodes when ever they take ________

A

alcohol

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28
Q

What is the prognosis of nodular sclerosis subtype of HL?

A

excellent (bcz low RS cells and high lymphocytes)

29
Q

Nodular sclerosis is usually EBV _________ (positive/negative)

A

negative

30
Q

Most patients of nodular sclerosis HL are __________ (young/old)

A

young

31
Q

Nodular sclerosis is diagnosed ________

A

early

32
Q

Key features of nodular sclerosis HL

A
  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
Q
The lacunar cells are
CD15 \_\_\_ (+/-)
CD30 \_\_\_ (+/-)
CD20 \_\_\_ (+/-)
A

CD15 +
CD30 +
CD20 -

34
Q

Nodular sclerosis HL usually presents as stage ___ or ____

A

usually stage I or II disease

35
Q

What are the microscopic features of Mixed cellularity otype of HL?

A

Diagnostic RS cells and mononuclear variants are plenty

Other cells - lymphocytes, eosinophils, plasma cells, macrophages

36
Q

What is the immunophenotype of mixed cellularity type of HL?

A

CD15+
CD30+
CD20-

37
Q

Reed-sternberg cells are usually ________ (infected/not infected) by EBV

A

infected (in 70% cases)

38
Q

Mixed cellularity is more common in _________ (males/females)

A

males

39
Q

Mixed cellularity HL is more associated with _________ (young/old)

A

old

40
Q

Mixed cellularity type HL has biphasic incidence. What does this mean?

A

It peaks in young age and again in adults older than 55

41
Q

People with Mixed cellularity type HL usually present in stage ___ or ____

A

III or IV

42
Q

Mixed cellularity type HL is associated w/ symptoms like _________ and _________

A

night sweats and weight loss

43
Q

Overall prognosis of Mixed cellularity type HL is _________

A

good

44
Q

What are the microscopic features of Lymphocyte Rich type HL

A

Rich of lymphocytes (T cells)

RS cells and mononuclear variants

45
Q

How do we differentiate between lymphocyte rich and lymphocyte predominance type?

A

Lymphocyte rich has RS cells and mononuclear variants whereas lymphocyte predominant type has popcorn cells
Immunophenotype is also different

46
Q

Compare the immunophenotype of lymphocyte rich and predominant HL.

A

Lymphocyte rich = 15+ and 30+

Lymphocyte predominance = 20+, 15- and 30-

47
Q

Lymphocyte rich type is associated with EBV in ____% of cases

A

40%

48
Q

Lymphocyte rich type has a _______ prognosis

A

good

49
Q

Lymphocyte rich HL occurs more commonly in ______ (males/females) and in ________ (young/old)

A

males

old

50
Q

Lymphocyte Depletion type is associated with EBV in ____% of the cases

A

90%

51
Q

Lymphocyte depletion type is associated with three factors. Name them

A

Older
HIV + regargless of age
Living in developing countries

52
Q

What are the microscopic features of lymphocyte depletion type of HL?

A

Very less lymphocytes

Abundance of RS cells

53
Q

What is the immunophenotype of lymphocyte depletion type of HL

A

CD15+

CD30+

54
Q

lymphocyte depletion type is associated with ________ stage

A

advanced stage

55
Q

Prognosis of lymphocyte depletion HL is _______ (better/worse) than other subtypes

A

worse

56
Q

What are the microscopic features of lymphocyte predominance type of HL

A

Lymphohistiocytic variant
Nodular infiltrate of lymphocytes
Macrophages

57
Q

WHat is the immunophenotype of LP type of HL

A

CD20+
BCL6+
CD15-
CD30-

58
Q

In some cases, LP type of HL may convert into ___________

A

B cell lymphoma

59
Q

Association of LP type of HL with EBV is ________ (common/uncommon)

A

uncommon

60
Q

LP type of HL is more common in ________ (male/female) and _________ (young/old)

A

males

young (less than 35)

61
Q

Which lymph nodes are commonly involved in LP type of HL?

A

Cervical and axillary

62
Q

LP type of HL can reccur and/but its prognosis is _________ (good/bad)

A

good

63
Q

What are the nodules made of in LP type of HL?

A

expanded B cells follicles
reactive B cells
dendritic cells

64
Q

WHat are the clinical features of HL in advanced stages?

A

Fever
Night sweats
Weight loss
Anergy

65
Q

What is anergy?

A

Cutaneous immune unresponsiveness due to low cell mediated immunity. RS cells make some factors that decrease T1H cells response

66
Q

What is the way of spreading of HL?

A

First node then spleen then liver and then marrow and others

67
Q

Cure rate if stage I and II =?

Cure rate if stage IVA or IVB =?

A

90%

disease free survival at 5 years is 60-70%

68
Q

What is the best treatment for HL so that no malignancy can develop?

A

Anti CD30 antibodies

Less genotoxic agents (other than radiotherapy and alkylationg agents)