Hodgkin's Lymphoma Flashcards

1
Q

What is the name of the giant lymphocytes seen in Hodgkin’s Lymphoma?

A

Reed-Sternberg (R-S)
together= Hodgkins/Reed-Sternberg (HRS)

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2
Q

What are Hodgkin Lymphomas?

A

neoplasms w/ heterogeneous mixture of non-neoplastic inflammatory cells & accessory cells

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3
Q

What are the common features to all Hodgkin lymphomas?

A
  • arise in single axial node/group
    • spread first to anatomically contiguous lymph nodes
  • unlikely to be extranodal
  • young adults
  • flow cytometry is negative
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4
Q

Is staging more important in Non-Hodgkin Lymphoma or Hodgkin Lymphoma?

A

Hodgkin

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5
Q

What are the genetic features of Reed-Sternberg cells?

A

B-lymphocytes of germinal or post-germinal center type

have genetic signature of B lymphocytes, but do not express most B cell-specific genes (including Ig)

aneuploid

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6
Q

What virus is associated with some Hodgkin Lymphomas? Which ones?

A

EBV

classical Hodgkin lymphoma

NOT nodular lyphocyte-predominant Hodgkin lymphoma

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7
Q

What is a risk factor for developing EBV-associated Classical Hodgkin Lymphoma (CHL)?

A

immunodeficiency

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8
Q

Compare & Contrast Hodgkin Lymphoma & Non-Hodgkin Lymphoma on the following variables:

nodal involvement

type of spread

Waldeyer ring & mesenteric node involvement

extranodal presentation

A
  • Hodgkin
    • single axial group of nodes
    • orderly contiguous spread
    • Waldeyer ring & mesenteric node rarely involved
    • Extranodal presentation rare
  • Non-Hodgkin
    • multiple peripheral nodes
    • non-contiguous spread
    • Waldeyer ring & mesenteric node commonly involved
    • Extranodal presentation common
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9
Q

What aspects of the provided images are indicative of Hodgkin Lymphoma?

A

mediastinal masses

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10
Q

What is Nodular Lymphocyte Predominant Hodgkin Lymphoma?

A

nodular proliferation large neoplastic B cells (LP cells) with spherical meshwork of follicular dendritic cells

inflammatory background of non-neoplastic small lymphocytes & histiocytes

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11
Q

What is the clinical presentation of a patient with NLPHL?

A
  • middle-aged male
  • peripheral lymphadenopathy
    • cervical, axial & inguinal
  • Indolent disease (no B symptoms)
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12
Q

The provided sample from a lymph node is indicative of what condition?

A

NLPHL

nodal architecture is distorted by multiple large nodules (black solid arrows)

compressed interfollicular zones (white)

nodules have “moth eaten” appearance (blue curved arrows)

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13
Q

What laboratory results would you expect to see in a patient with NLPHL?

A

Normal CBC; no leukemic phase

Serum LDH or beta-2-microglobin are rarely elevated

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14
Q

What is the treatment & prognosis of NLPHL?

A
  • Treatment
    • depends on stage (chemo & radiation)
  • Prognosis
    • fairly frequent relapses, but responsive to therapy
    • 10 yr survival >80%
    • death may be due to refractory disease or development second malignancy
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15
Q

What type of malignancy is shown in the provided image?

A

NLPHL

lymph node is large & multinodular

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16
Q

How would you differentiate the microscopic appearance of a lymph node from a patient with NLPHL from a patient with usual reactive lymphoid follicles?

A
  • NLPHL
    • expansile nodules are larger
    • composed mainly small lymphocytes & histiocytes
    • no eosinophils or plasma cells
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17
Q

What aspects of the provided slides are indicative of NLPHL?

A

complete or partial effacement of lymph node architecture by infiltrate

LP scattered throughout & only about 1% of all cells

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18
Q

What type of cell is shown in the provided image? It makes up what percent of cells seen in sample form NLPHL?

A

LP “popcorn cell” - only 1%

multilobated nuclei with vesicular chromatin & multiple small nucleoli

multinucleated or mummified cells

round nuclei without multilobation

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19
Q

NLPHL is positive & negative for what immunophenotypes?

A

Positive: CD45, CD20

Negative: CD30, CD15

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20
Q

Is flow cytometry usually positive or negative in NLPHL?

A

negative

large neoplastic cells typically lost/overlooked b/c are so few in number

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21
Q

What is the postulated normal counterpart of the neoplastic cells seen in NLPHL?

A

germinal center B lymphocytes

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22
Q

What is the definition of Classical Hodgkin Lymphoma?

A

tumor comprised of scattered malignant lymphocytes in a mixed inflammatory background (non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts & collagen fibers)

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23
Q

What is the difference in the definition between CHL & NLPHL?

A

CHL: background of non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts & collagen

NLPHL: background inflammation of only small lymphocytes & histiocytes

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24
Q

CHL is what percentages of all Hodgkin lymphomas?

Lymphomas overall?

A

90% all Hodgkin

30% lymphomas overall

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25
Q

CHL most commonly affects what age groups?

A

Bimodal

15-35 & second peak later in life

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26
Q

CHL is more commonly seen in males, except what type?

A

nodular sclerosis

27
Q

What sites are most commonly involved in CHL?

A

lymph nodes: cervical (75%)

mediastinal, axillary, & paraaortic

primary extranodal involvement & BM involvement is rare

NO leukemic phase

28
Q

Mediastinal involvement is more common in what type of CHL?

A

nodular sclerosis type

29
Q

Abdominal & splenic involvement is more common in what type of CHL?

A

mixed cellularity type

30
Q

What are the key features for a cell to be diagnosed as a Reed-Sternberg cell? What about Hodgkin cells?

A

at least 2 nucleoli in two separate nuclear lobes

if mononuclear → Hodgkin cell

together are called HRS

31
Q

What cell type is shown in the provided images?

A

Reed-Sternberg

at least 2 nucleoli in two separate nuclear lobes

32
Q

What are the reddish cells with the condense chromatin seen in CHL?

A

“mummified” cells

33
Q

What are the four histological subtypes of CHL?

A

Nodular sclerosis CHL (NSCHL)

Lymphocyte rich CHL (LRCHL)

Mixed cellularity CHL (MCCHL)

Lymphocyte depleted CHL (LDCHL)

34
Q

What are the immunophenotypes seen in all types of CHL?

A

Positive: CD30, CD15 (most)

Negative: CD20, CD45

35
Q

What is the most common CHL seen in the US?

A

nodular sclerosis CHL

36
Q

Nodular Sclerosis CHL most commonly occurs in what demographics?

A

15-35

white

37
Q

Nodular sclerosis CHL is seen most commonly in what lymph node?

A

mediastinal (80%) & cervical

38
Q

What is the clinical presentation of a patient with Nodular Sclerosis CHL?

Treatment?

Prognosis?

A

bulky lymphadenopathy (50%)

B symptoms (40%)

Treatment: chemo and/or radiation

good prognosis overall

39
Q

Describe the gross morphology of the tumor seen in nodular sclerosis CHL

A

enlarged, matted lymph node with nodules & fibrous bands around the nodules

40
Q

The provided image is a sample from a patient with what type of CHL?

A

Nodular sclerosis CHL

effacement of lymph node architecture by neoplastic nodules that are surrounded by dense, fibrous bands

41
Q

The provided histological slide is from a sample of what type of CHL tumor? What features helped you to identify this?

A

Nodular Sclerosis CHL

  • Most cells are variants of HRS called lacunar cells - appear to by lying in clear area or “lake”
  • folded nuclear membranes, less conspicuous nucleoli & lots of clear cytoplasm
  • Classic HRS are also seen
42
Q

What is the definition of Mixed cellularity CHL (MCHL)?

A

CHL with classic RS & Hodgkin cells in highly mixed inflammatory background in a diffuse or interfollicular pattern without nodules or fibrosis

43
Q

EBV is present in what percent of Nodular Sclerosis CHL?

A

20%

44
Q

EBV is present in what percent of Mixed Cellularity CHL?

A

75%

45
Q

What other virus predisposes patients to developing EBV-associated MCHL?

A

HIV - predisposes development of EBV-associate MCHL

46
Q

What is the most common CHL in underdeveloped countries?

A

MCHL

47
Q

MCHL is most commonly seen in what demographics?

A

38yr

M:F = 2:1

48
Q

MCHL is most commonly found in what sites?

A

lymph nodes, supraclavicular MC

mediastinal is uncommon

49
Q

Does NSCHL or MCHL have a better prognosis?

A

MCHL is slightly worse

50
Q

The provided histological slide is a sample from what type of CHL?

A

MCHL

  • complete or partial effacement of lymph node architecture
  • H-RS readily identifiable
  • background is mixture of small lymphocytes, plasma cells, histiocytes, eosinophils, & neutrophils
  • mild to moderate interstitial fibrosis may be present, but NO nodular collagen bands & NO thickening of capsule
51
Q

What is the definition of Lymphocytes rich CHL?

A

predominance of small reactive lymphocytes associated with RS & Hodgkin cells

52
Q

How can you differentiate Lymphocyte Rich CHL from MCHL?

A

granulocytes & plasma cells are infrequent in Lymphocyte Rich CHL

53
Q

Lymphocyte-Rich CHL is most commonly seen in what demographics?

A

middle age (43)

M:F = 2:1

54
Q

What sites are most commonly involved with Lymphocyte-Rich CHL?

A

Lymph nodes -Waldeyer ring (throat) is common

peripheral nodal involvement is typical (supradiaphragmatic)

mediastinal involvement is uncommon

55
Q

What is the clinical presentation of a patient with Lymphocyte-Rich CHL?

Prognosis?

A

Only 10% have B symptoms

typically lack bulky disease

good to excellent prognosis

56
Q

What are the two type of growth patterns seen in Lymphocyte-Rich CHL?

A
  • Nodular variant
    • vague nodularity
    • mantle zone small lymphocytes with underlying meshwork follicular dendritic cells
  • Diffuse variant
    • similar to above but with diffuse replacement of lymph node
57
Q

The provided histological slides are samples taken from what type of CHL tumor?

A

Lymphocyte-Rich CHL

Lymph node replaced by large, vague nodularity

HRS scattered among small lymphocytes

small number histiocytes

ABSENT- plasma cells, eosinophils, neutrophils

58
Q

What is the ancillary test for Lymphocyte-Rich CHL?

A
  • Immunophenotype & genetic tests - HRS have features like CHL
    • strong (+) CD15 & CD30
  • Background follicular dendritic network & T-cell rosettes more like NLPHL
    • histiocytes also stain CD15 (+) but are more pale
    • activated immunoblasts are CD30 (+) but are smaller than neoplastic cells
59
Q

What percent of Lymphocyte-Depleted CHL are EBV positive?

A

50%

60
Q

Lymphocyte-Depleted CHL is most commonly seen in what demographics?

A

4th decade or older

61
Q

What is the likely pathogenesis of Lymphocyte-Depleted CHL?

A

likely represents progression from other types of CHL

62
Q

What is the clinical presentation of a patient with Lymphocyte-Depleted CHL?

A

B symptoms are frequent

lymphadenopathy

63
Q

What are the most common sites of involvement for Lymphocyte-depleted CHL?

A
  • Lymph nodes
    • retroperitoneal or abdominal
    • peripheral lymph nodes
    • abdominal organs
    • bone marrow
64
Q

The provided histological slides are samples taken from what type of CHL tumor?

A

Lymphocyte-depleted CHL

  • Lymph node is diffusely effaced with depletion of small lymphocytes & scant granulocytes and granulocytes