Hodgkin Lymphoma Flashcards

1
Q

what is Hodgkin Lymphoma?

A

it’s a unique form of B-cell lymphoma with an excellent prognosis. Cells have largely lost their B-cell phenotype.

HL arises from lymphoid tissue

Reed-Sternberg cells are characteristic to an HL lymph node biopsy

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2
Q

what are Reed-Sternberg cells?

A

characteristic to an HL - this cell is not found in other types of lymphoma

***when present, true RS cells are diagnostic of HL

a binucleated cell with centrally located nucleoli giving it a classic “owl’s eye” appearance

go look at a picture of it

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3
Q

what type of cell do RS cells originate from?

A

germinal or post-germinal B cells

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4
Q

what immunophenotype markers are RS cells positive for?

A

CD15 and CD30

they don’t stain positive for CD 20! this weird loss of the B-cell phenotype makes HL unique among other lymphomas

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5
Q

what does an H&E of HL show?

A

a reactive cell population – the reactive cell population makes up the bulk of the “tumor,” even though the reactive cells are totally benign

lymphocytes are most commonly found but eosinophils may also be present

RS cells and their variants only make up a small percentage of the total number of cells present

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6
Q

what are the 5 histological subtypes of HL?

A
  1. nodular sclerosis
  2. mixed cellularity
  3. lymphocyte-rich
  4. lymphocyte-depletion
  5. lymphocyte-predominance HL (not a classic subtype)
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7
Q

what is nodular sclerosis HL?

A

Classic RS cells or lacunar cells (CD15+, CD30+ immunophenotype)

lacunar cells are variant RS cells

fibrous bands divide the lymph nodes into nodules

presents with cervical, supraclavicular or mediastinal lymphadenopathy

good prognosis!

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8
Q

what population in nodular sclerosis HL most common in and what’s its overall prevalence?

A

60% prevalence

M=F

more common in adolescents and young adults

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9
Q

what is mixed cellularity HL?

A

there’s an abundant background infiltrate of all different kinds of cells like eosinophils, lymphocytes, plasma cells, macrophages

these cells are recruited by IL-5 which is secreted by the RS in this subtype of HL

systemic involvement

over half of the patients with this subtype present with advanced-stage disease, but the prognosis overall is still good

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10
Q

what population in mixed cellularity HL most common in and what’s its overall prevalence?

A

25% prevalence

M>F

biphasic incidence, with one peak in young adulthood and another in adults over the age of 55

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11
Q

what is lymphocyte-rich HL?

A

has classic RS cells

has lymphocytes form background inflammatory infiltrate

present with early-stage localized lymphadenopathy and has a great prognosis!

B symptoms are rare

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12
Q

what’s lymphocyte-rich HL overall prevalence?

A

5% prevalence

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13
Q

what is lymphocyte-depletion HL?

A

CD15+, CD 30+ RS cells

few inflammatory cells in the background

necrosis and fibrosis present

more aggressive; systemic symptoms common

usually presents with advanced stage disease and prognosis is less favorable.

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14
Q

what is the prevalence of lymphocyte-depletion HL?

A

5%

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15
Q

what is lymphocyte-predominance HL?

A

classic RS cells are rare; instead there’s multilobed CD20+ “popcorn cell

tons of small B-cells and macrophages in histology

usually limited to cervical or axillary lymphadenopathy

B symptoms are rare

relapsing course; good prognosis!

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16
Q

what’s the prevalence of lymphocyte-predominance HL?

A

5%

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17
Q

what’s the most common type of HL?

A

nodular sclerosis

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18
Q

what are lacunar cells?

A

variant RS cells present in nodular sclerosis HL

lacunar cells have delicate nuclei that appear to sit in clear “lakes” (lacunae)

this is because formalin fixation of the tissue causes the cell cytoplasm to retract, leaving an empty space around the nucleus of the cell

19
Q

what’s the immunophenotype of lacunar cells?

A

CD15+, CD30+

the exact same as classic RS cells!

20
Q

what’s the least common type of HL?

A

lymphocyte-depletion HL

21
Q

what population in lymphocyte-deletion HL most common in?

A

typically occurs in older adults and in HIV positive patients

22
Q

what population is lymphocyte predominance HL most common?

A

usually males under 35 years old

23
Q

what makes lymphocyte-predominance hL a separate entity from classical HL?

A
  1. classic RS cells are scare

there are neoplastic B cells that express J chains, epithelial membrane antigen (EMA), CD 20, and CD 45 on their cell surface – classic RS cells have CD15 and CD30 on its surface

  1. lymphocyte- predominance HL has its very own malignant cells, often referred to as the popcorn cell

they express CD20 and are variant RS cells

24
Q

Which neoplastic cells are present in lymphocyte-predominance HL and which markers do they express?

A

Lymph node biopsy in lymphocyte-predominance HL shows proliferating B cells that express J chains, epithelial membrane antigen, CD 20 and CD 45 on their cell surface.

macrophages are also usually present. Another type of malignant cell found in in this subtype is the so-called CD20+ popcorn cell, which is a variant of the classic RS cell.

25
Q

what’s the average age of diagnosis of HL?

A

35 is the average age of diagnosis

but there’s actually 2 peaks, it’s one of the most common cancers of adolescents and young adults; however, it also occurs in the aged

26
Q

what’s the clinical presentation of HL?

A

HL has a vague presentation and can easily be misdiagnosed

non-tender, enlarged cervical lymph nodes are often the first presenting sign

lymphadenopathy may be present in the axilla and supraclavicular region

1/2 of patients also have mediastinal lymphadenopathy

can also have fevers, night sweats, weight loss = B symptoms

27
Q

how does HL usually present?

A

the most common sign is cervical, supraclavicular, or axillary lymph node enlargement

mediastinal lymphadenopathy is present in about 50% of patients at the time of diagnosis

approximately 30% of patients may also report so-called B symptoms (weight loss, fever, night sweats).

28
Q

what are the risk factors for HL?

A

Epstein-Barr Virus (EBV)

primarily associated with the lymphocyte- depletion and mixed cellularity subtypes of classical HL

in vitro experiments have shown that the virus can prevent germinal B cells from undergoing apoptosis by interacting with tyrosine-kinase receptors or modulating host DNA but we don’t know if EBV for sure plays a pathogenic role in HL

29
Q

what is the staging system used for HL?

A

the Ann Arbor Staging system - the same as NHL!

30
Q

what is stage 1 HL?

A

Involves 1 single lymph node or lymphoid structure

31
Q

what is stage 2 HL?

A

Involves >2 lymph nodes on the same side of the diaphragm

32
Q

what is stage 3 HL?

A

involves lymph nodes or lymphoid structures on both sides of the diaphragm

33
Q

what is stage 4 HL?

A

involves extranodal sites including liver and bone marrow

34
Q

what does the A,B and E designation in the Ann Arbor staging system mean?

A

A – No symptoms

B – Weight loss of >10% in last 6 months; fever > 100F (38C) in last month; night sweats in last month

E – Extranodal involvement (excluding liver and bone marrow)

35
Q

what test is best for identifying how far the lymphoma has progressed?

A

imaging

a chest x-ray may show evidence of mediastinal lymphadenopathy

ultimately, a CT chest/abdomen/pelvis is needed and may even be supplemented with the PET scan

a bone marrow biospy can identify Stage IV disease

36
Q

how do you determine the treatment plan for HL?

A

the stage does not just predict prognosis but also determines the best treatment modality

37
Q

how can you treat HL?

A

early localized disease may be treated with radiotherapy only

the most common regimen for HL consists of doxorubicin, belomycin, vinblastine, and dacarbazine (ABVD)

a 12-week course of Standford V that includes radiotherapy is also often used

38
Q

what are the new biological therapies being used to treat HL?

A

Rituximab, a chimeric (mouse/human) monoclonal anti-CD20 antibody, has been given to patients with classical HL and lymphocyte-predominance HL with some success

patients with lymphocyte-predominance subtype require long-term followup to ensure a relapse is detected early

39
Q

how is HL typically treated?

A

Low stage or localized may be managed with wide-field radiotherapy alone

chemotherapy and chemoradiation are the mainstay for most stages of HL

half of the refractory cases can be cured with autologous bone marrow transplantation

40
Q

what’s the prognosis for HL?

A

pretty good honestly, especially in early stages of HL

5-year survival rate for HL is >70% in most instances

in about 50% of non-responders, autologous bone marrow transplant induces remission

41
Q

what’s the negative side effect of HL treatment?

A

chemotherapeutic agents remain toxic to healthy cells

HL patients are now living long enough to experience significant side effects, including secondary malignancies, decades after their initial treatment

42
Q

classic RS cells express which cell surface markers?

A

CD15 and CD30

43
Q

which microorganisms is found in up to 40% of Hodgkin Lymphoma (HL) cases?

A

Epstein-Barr virus

its DNA has been found in RS cells in about 40% of cases

however, it remains unclear whether this virus plays a pathogenic role

44
Q

A patient with cervical and para-aortic lymph node enlargement also complains of regular night sweats in the past two weeks. Excisional lymph node biopsy confirms HL. Bone marrow biopsy and liver ultrasound are clear. What is the correct stage in the Ann Arbor system?

A

IIIB

Stage III is defined by lymphadenopathy at both sides of the diaphragm (cervical and para- aortic lymph nodes in this case)

patient also has B symptoms