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Pathology 1 Block 3 > WBC & Lymph nodes, Part 5: 
Lymphomas > Flashcards

WBC & Lymph nodes, Part 5: 
Lymphomas Flashcards

1
Q

lymphomas are classified into what 2 types?

A
  1. Non- Hodgkin Lymphoma (NHL)
  2. Hodgkin Lymphoma (HL)
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2
Q

what are the B-cell lymphomas found in the non-Hodgkin Lymphoma ?

A
  1. Follicular Lymphoma
  2. Diffuse Large B-cell Lymphoma (DLBCL)
  3. Burkitt Lymphoma
  4. Mantle cell Lymphoma
  5. Marginal zone Lymphoma
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3
Q

what are the T-cell lymphomas found in the non-Hodgkin Lymphoma ?

A

Anaplastic Large-cell Lymphoma

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4
Q

What are the sub-types found in Hodgkin Lymphoma?

A
  1. Nodular sclerosis
  2. Mixed cellularity
  3. Lymphocyte rich
  4. Lymphocyte depletion
  5. Lymphocyte predominant
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5
Q

Follicular Lymphoma

  1. how common is it?
  2. what is its incidence?
  3. what is its pathogenesis?
A
  1. common type of lymphoma
  2. middle aged, males and females
  3. Pathogenesis:
  • t(14;18) causing overexpression of BCL-2 so it causes inhibition of apoptosis, therefore these cells keep proliferating
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6
Q

Follicular lymphoma Labs

  1. CBC?
  2. Lymph node biopsy?
  3. What organ is mainly involved?
  4. What other organs are involved?
  5. Immunophenotype of tumor?
  6. What other investiagion can be used?
A
  1. normal
  2. loss of cell architecture, there is follicle-like pattern, stains positive for BCL-2
  3. Bone Marrow
  4. Liver and Spleen
  5. tumor expresses CD10, CD19, CD20,
  6. CT scan
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7
Q

what is this?

A

Follicular lymphoma

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8
Q

what will you notice about a microscopy of a lymph node with follicular lymphoma?

A
  • numerous follicle-like structures
  • absence of germinal centers
  • absence of tingible body macrophages
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9
Q

what is this?

A

follicular hyperplasia

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10
Q

Follicular Lymphoma

what are the clinical features?

is it curable?

what is the median survival rate?

A
  • painless generalized lymphadenopathy
  • about 10% of patients present with B symptoms (fever, drenching night sweats, or loss of 10% of the body weight)
  • disease usually is widespread at presentation, with involvement of multiple lymph node, liver, and spleen
  • bone marrow is involvement seen
  • incurable, but has an indolent course
  • median survival: 7 to 9 years
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11
Q

Follicular Lymphoma

into what can follicular lymphoma transform?

what is the prognosis after transformation?

A

to “diffuse large B cell lymphoma”

poor prognosis with transformation

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12
Q

Diffuse large B cell lymphoma

  1. on who is it seen?
  2. how aggresive is it?
  3. what may it involve?
  4. what are its special sub-types?
  5. what are the molecular genetics at play here?
A
  1. any age group, but mostly middle-age
  2. aggressive
  3. may involve ‘extra-nodal’ sites (example: GIT, liver, brain)
  4. Special subtypes:

Immunodeficiency associated (bone marrow transplantation/advanced HIV infection)

Primary Effusion Lymphoma (PEL). Seen mostly in advanced HIV infection

  1. dysregulation of BCL-6 (this is normally required for the formation of normal germinal centers)
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13
Q

Diffuse large B cell lymphoma

CBC?

Bone Marrow?

Serology?

LDH?

CT scan?

flow cytometry?

A

CBC= normal

Bone Marrow = involved in late disease

Serology = HIV

LDH = increased due to increased tumor burden

CT scan = abdominal lymph nodes

flow cytometry = Diagnosis is based on tissue biopsy:

  • Lymph node/tissue morphology
    • presence of large lymphoid cells
    • immunophenotype: CD19, CD20
    • surface Ig is seen
    • negative for TdT
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14
Q

what is this?

A

Diffuse large B-cell lymphoma: lymph node contains large cells with enlarged nuclei, open chromatin, and prominent nucleoli

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15
Q

what does this aspiration cytology reveal?

does this stain positive for? what will it stain negative for?

A

Primary Effusion Lymphoma (PEL) with large pleomorphic cell

stain positive for immunoglobulin

stain negative for cytokeratin

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16
Q

Diffuse Large B cell Lymphoma

what is the clinical presentation?

what do you use for therapy?

where can nodes arise as well?

when is bone marrow involved?

A
  • rapidly enlarging lymph nodes at single nodal site or extra nodal site where aggressive tumors appear
  • adjuvant therapy with anti-CD20 antibody
  • extra nodal: GIT, skin, brain
  • bone marrow involvement is late
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17
Q

Burkitt Lymphoma

what are the 3 types?

with what diseases are each associated?

A
  1. African (endemic): malaria or EBV
  2. Sporadic (non endemic): large abdominal masses
  3. Aggressive form: associated with HIV
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18
Q

Burkitt Lymphoma

African (endemic) type:

  1. in what age group do we see it?
  2. what are the clinical features we find in it?
  3. what immunophenotype do we see in the tumor?
  4. what translocation do we see here?
A
  1. children, young adult
  2. Clinical features:
    - extra-nodal
    - common location: mandible, abdominal viscera (kidney, ovaries, adrenal)
    - aggressive, but responds to chemotherapy
  3. CD19, CD20, B-cell expression of IgM
  4. t(8; 14): myc gene on chromosome 8. The myc (transcriptional promoter) is relocated adjacent to strong Ig promoter and enhancer which drive increased myc expression
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19
Q

what is this?

A

Burkitt’s lymphoma African (endemic) type

20
Q

Burkitts Lymphoma

CBC?

Bone Marrow?

The diagnosis for this disease is based on what 2 things?

A

CBC = normal

B. marrow = barely involved

1) tissue biopsy
2) karyotyping

21
Q

Burkitts Lymphoma

African (endemic) type:

How do you see it under the microscope?

involved tissues will show what?

A

as starry sky pattern

diffuse infiltrate of intermediate-sized lymphoid cells

22
Q

what is this?

A

burkitts lymphoma - starry sky pattern

23
Q

what is this?

A

Mantle Cell Lymphoma

24
Q

In mantle cell lyphoma, tumors will resemble what?

what can be expected in a lab report for mantle cell lymphoma:

CBC

Bone Marrow

Lymph node biopsy

Immunophenotype

A

the mantle zone B lymphocytes

CBC = normal

Bone Marrow = involved

Lymph node biopsy = loss of arquitecture, few reactive germinal centers, surrounded by tumor cells

Immunophenotype =

  • CD 19/20/5 positive
  • CD 23 negative
  • cyclin D1 expressed
25
Q

why is it important that CD23 be tested for in Mantle cell lymphoma?

What are the clinical features of Mantle cell Lymphoma?

how curable is it?

A

because if negative, we know it is not CLL or SLL

C/F:

  • painless lymphadenopathy
  • splenomegaly
  • Extranodal: Waldeyer ring, and GIT
    • GIT = lymphmatoid polyp

by the time the pt seeks treatment it is stage 4 (rarely curable)

26
Q

what is this?

what can be noted here?

A

Mantle Cell Lymphoma

LN Biopsy: note the distribution of cyclin D1 positivity around germinal centers.

27
Q

what is this?

what can be noted here?

A

Mantle Cell lymphoma

Lymphmatoid polyp: Cyclin D1 positivity (dark brown).

28
Q

what is Marginal zone lymphoma?

what are the clinical findings for marginal zone lymphoma?

A
  • a distinct B-cell neoplasms with variable clinical features

C/F:

  1. extra nodal marginal zone MALToma
    * stomach, orbit, intestine, lung, thyroid, salivary gland, bladder, CNS
  2. nodal marginal zone lymphoma
  3. splenic marginal zone lymphoma
29
Q

Extranodal involvement in Marginal Zone Lymphoma is seen when?

extranodal area involvement will remain involved for how long?

what is the immunophenotype markers that we look for in marginal zone lymphoma?

A
  • in areas involved in chronic inflammation by autoimmune or infectious etiology
    • (Sjörgen syndrome, Hashimoto’s thyroiditis, Helicobacter pylori)
  • long time
  • B cell markers: CD19, CD20
30
Q

how do MALTomas begin as in Marginal Zone Lymphoma?

A

MALTomas begin as polyclonal immune reactions and later acquire an initiating mutation in the B-cell clone

  • as these cells divide more mutations are gained which will make these cells autonomous
  • NF-kB activation promotes growth and survival
31
Q

what is this?

A

Marginal Zone Lymphoma

32
Q

eradication of Helicobacter pylori in Marginal Zone Lymphoma will results in what?

A

tumor regression in gastric MALTomas

33
Q

what is this?

what immunostain is being used here?

A

H. pylori infection from gastric MALToma

CD19

34
Q

Anaplastic Large-cell Lymphoma is what type of lymphoma?

why does it happen?

A

it is a T cell lymphoma

re-arrangement in ALK gene (2p23) leading to JAK/STAT pathway activation

35
Q

what can we expect in a skin biopsy in Anaplastic Large-cell lypmhoma?

what can we expect in a Lymph Node biopsy in Anaplastic Large-cell lypmhoma?

why is the prognosis good?

A
  1. infiltrates of large anaplastic cells with horseshoe nuclei (hallmark cells)
  2. loss of architecture, large anaplastic cells, “hallmark” or “doughnut” cells positive for CD30 (Ki-1)
  3. because it is a T-cell malignancy
36
Q

what is this?

A

Anaplastic Large-cell Lymphoma in Lymph node: loss of architecture, note giant cells (hallmark cells)

37
Q

what is this?

A

Lymph node Anaplastic Large-cell Lymphoma

38
Q

what characterizes Hodgkins lymphoma?

on who does it happen more oftenly?

how do you describe the Reed-Sternberg cell?

A

Reed-Sternberg cell: residing in a mixed infiltrate of non-neoplastic cells

mostly young adults

Reed-Sternberg cell has a bilobed nucleus with prominent eosinophilic nucleoli separated by a clear space from the thickened nuclear membrane

39
Q

what is the pathogenesis of Hodgkin’s Lymphoma?

A
  • IgG genes of Reed Sternberg cells have undergo V(D)J recombination and somatic hypermutation in germinal/post-germinal center in B cells
  • RS cell fail to express most B-cell specific markers
    • NF-kB activation is seen
      • helps these abnormal RS from being removed through apoptosis
  • RS cells produces cytokines (IL-5, IL-10, 1L-13, & TGF-β),
    • which creates a reactive response in the surrounding cells
40
Q

what are the classifications of Hodgkins lymphoma?

what is the prognosis for each?

A
  1. Nodular sclerosis (good prognosis)
  2. Mixed cellularity
  3. Lymphocyte rich (good prognosis)
  4. Lymphocyte depletion (poor prognosis)
  5. Lymphocyte predominant
41
Q

what is the most common form of Hodgkin’s lymphoma?

A

nodular sclerosis Hodgkin’s lymphoma

42
Q

nodular sclerosis hodgkin lymphoma involves usually what areas?

what Reed Steinberg cell variant do we see here?

How does nodular sclerosis hodgkin lymphoma look like?

A
  • frequent involvement of the lower cervical, supraclavicular, and mediastinal lymph nodes
  • ‘lacunar cell’
  • fibrous bands, going around the lymph node into nodular areas, ‘lacunar RS cells’ are seen in a reactive background composed of T-cells, eosinophils, plasma cells, macrophages
43
Q

what can we see if we do immunophenotyping in nodular sclerosis hodgkin lymphoma?

what areas are the most commonly involved in nodular sclerosis hodgkin lymphoma?

A

CD15, CD30 positive

lower cervical, supra clavicular and mediastinal lymph nodes

44
Q

what is this?

A

Hodgkin lymphoma Lymph Node Biopsy: Lacunar variant cells (arrow) are seen within spaces created by tearing away of the wispy cytoplasm during tissue sectioning

45
Q

what is the clinical course of hodgkin lymphoma?

A

Clinical course:

  • painless lymph node enlargement of neck, supraclavicular, axilla, mediastinal lymph nodes in 50% at the time of diagnosis
  • fevers, night sweats, and/or weight loss
    • fever: Pel-Epstein fever
      • fevers persist for days to weeks, followed by afebrile intervals and then recurrence
46
Q

Hodgkin Lymphoma long term survivors of chemotherapy, and radiotherapy have an increased risk for whatsecond malignancy?

A

AML in the setting of MDS, lung cancers, and breast cancers

Pathology 1 Block 3 (7 decks)

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