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Hematology > Hematology Week 3: Lymphoma II > Flashcards

Hematology Week 3: Lymphoma II Flashcards

1
Q

Lymphoma Main Categories

A
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2
Q

Follicular Lymphoma Clinical Features

4 listed

A
  • Painless generalized lymphadenopathy is common
  • incurable and indolent clinical course except when transformed
  • histologic transformation occurs in up to half of the cases
  • Diffuse large B-cell lymphoma is the most common type of transformation but can also become Hodgkins Lymphoma
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3
Q

Most common indolent type of Non-Hodgkin Lymphoma in the US

A

Follicular Lymphoma

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4
Q

Follicular Lymphoma Morphological Features

5 listed

A
  • Nodular growth pattern
  • closely arranged neoplastic follicles
  • Neoplastic cells include:
  • Small cleaved lymphocytes (centrocytes)
  • Large lymphocytes (centroblasts)
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5
Q

Follicular Lymphoma Cells arise from?

A

Germinal Center B-cells

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6
Q

Follicular Lymphoma Neoplastic Cells

2 listed

A
  • Small cleaved lymphocytes (Centrocytes)
  • Large Lymphocytes (Centroblasts)
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7
Q

Follicular Lymphoma Histology

A

look like germinal center cells

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8
Q

Neoplastic vs Reactive Follicles: Reactive Follicle Features

4 listed

A
  • Reactive Follicle:
  • Intact mantle zone
  • Many ‘tingible body’ macrophage
  • polarization is present
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9
Q

Neoplastic vs Reactive Follicles: Neoplastic Follicle Features

3 listed

A
  • Absent or thin Mantle Zone
  • Lack of ‘tingible body’ macrophages
  • lack of polarization
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10
Q

Identify

A

Reactive Follicle

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11
Q

Tingible Body Macrophages

A

beig macrophages with lots of debris

present in a reactive follicle

Absent in neoplastic follicle

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12
Q

Follicular Lympha Flow Cytometry

A
  • Small cells
  • CD19+ CD20+ and CD10+ (germinal center like)
  • Monoclonal so either kappa or lambda
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13
Q

Follicular Hyperplasia Flow Cytometry

A
  • small cells
  • few CD10+ germinal center cells
  • B cells are not monoclonal
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14
Q

Follicular Lymphoma Genetics

A

t(14;18)(q32;q21)

  • present in 90% of follicular lymphomas
  • Balanced reciprocal translocation
  • BCL2 oncogene on chromosome 18 is juxtaposed to IGH gene on chromosome 14
  • Results in upregulation of BCL2 gene
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15
Q

Follicular Lymphoma specific mutation

A
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16
Q

Follicular Lymphoma Genetic testing

A

t(14;18)(q32;q21) can be seen in karyotype

or FISH

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17
Q

Follicular Lymphoma Pathophysiology

3 listed

A

consequences of BCL2/IGH rearrangement

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18
Q

Follicular Lymphoma Immunohistiochemistry

A
  • Reactive follicles never express BCL2
  • Only neoplastic germinal centers express BCL2 in 90% of them
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19
Q

Mantle Cell Lymphoma prevalence

A

2.5% of Non-Hodgkin Lymhoma

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20
Q

Mantle Cell Lymphoma Clinical Presentations

A
  • commonly present with painless lymphadenopathy
  • Frequent extranodal involvement including BM, spleen, liver and GI tract
  • intestinal involvement may present with polyps (lymphomatoid polyposis)
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21
Q

Mantle Cell Lymphoma Prognosis

4 listed

A
  • Very aggressive
  • Poor prognosis
  • median survival 3-4 years
  • Currently incurable
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22
Q

Mantle Cell Lymphoma Cells come from?

A
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23
Q

Mantle Cell Lymphoma Morphologic Features

A
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24
Q

Mantle Cell Lymphoma Immunophenotype

4 listed

A
  • CD19+
  • CD20+
  • CD5+ (usually T cell marker)
  • Cyclin D1+
25
Q

Mantle Cell Lymphoma Genetics

A

t(11;14)(q13;q32)

26
Q

Mantle Cell Lymphoma Pathophysiology

A
  • CCD1 (Cyclin D1) gene juxtaposed to the IGH gene resulting in upregulation of the gene
  • The upregulated CCND1 gene results in overexpression of cyclin D1
27
Q

Mantle Cell Lymphoma Genetic testing

A
  • Karyotype
  • FISH
28
Q

Mantle Cell Lymphoma Immunohistochemistry

A
29
Q

Role of Cyclin D1 in cell cycle

A

passes G0 to S

30
Q

Marginal Zone Lymphoma Follicle Histology

A

Arise from memory B cells in the marginal zone

31
Q

Marginal Zone Lymphoma Morphology

A
32
Q

MALT Lymphoma Associations

2 listed

A
33
Q

Helicobacter pylori associations with lymphoma

A
34
Q

MALT Lymphoma Clinical Course

A
35
Q

MALT Lymphoma Immunophenotype

4 listed

A
  • CD19+
  • CD20+
  • CD5-
  • CD10-
36
Q

Burkitt Lymphoma Types

3 listed

A
  • Endemic in Africa all EBV+
  • Sporadic
  • Immunodeficiency-related (HIV)
37
Q

Burkitt Lymphoma Histology

A

Identical histology in all types Starry-sky

38
Q

Burkitt Lymphoma Immunophenotype

4 listed

A
  • CD20+
  • CD10+
  • BCL6+
  • BCL2-
39
Q

Burkitt Lymphoma Genetics

3 listed

A
  • t(8;14) in 80%
  • t(2;8) or t(8;22) in 20%
40
Q

Burkitt Lymphoma Endemic type and lymphoma belt of Africa

A
41
Q

Fastest growing tumor

A

Burkitt Lymphoma

42
Q

Burkitt Lymphoma Molecular Pathogenesis

A

t(8;14)(q24;32) in 80% of cases

  • MYC gene on chromosome 8 is juxtaposed to the IGH locus on chromosome 14
  • The breakpoints in IGH locus in endemic occurs within or in proximity to JH region
  • The breakpoints in IGH locus in sporadic and HIV-associated Burkitt Lymphomas involve class switching regions
43
Q

BurkitBurkitt Lymphoma genetic testing

A

Karyotype and FISH

44
Q

Burkitt Lymphoma Pathophysiology

A

All translocations result in MYC gene upregulation and the consequences are:

  • promote cell cycle entry
  • block differentiation
  • Stimulate cell proliferation
  • increase cellular metabolism
  • Induce apoptosis in the absence of adequate growth support
45
Q

Clinical & Diagnostic utility of detection of specific chromosomal rearrangements

3 listed

A
46
Q

The significance of distinction of subtypes of B-cell lymphoma

A
47
Q

Antigen receptor gene rearrangements

A
48
Q

Light and Heavy chain rearrangements

A
  • this process involves chromosomal breaking and rejoining and errors can occur during this process
  • The antigen receptor genes serve as a second set of tumor markers in lymphoid neoplasms
  • Antigen receptor gene rearrangements in lymphoid cells occur often before clonal expansion
49
Q

Clinical and diagnostic utility of IGH gene rearrangements

3 listed

A

PCR can identify clonal populations

50
Q

Polyclonal vs monoclonal populations capillary electrophoresis

A
51
Q

Question 1

A

Mantle Cell Lymphoma

52
Q

Question 2

Mantle Cell Lymphoma

A

C

  • CD10+
  • CD20+
  • CD5+
  • Cyclin D1+
53
Q

Summary of Selected Mature B Cell Lymphomas

A
54
Q

Indolent Lymphomas Treatment

A
  • incurable in an advanced stage
  • Treat only symptomatic Disease
  • Rituximab + Chemotherapy
55
Q

Aggressive Lymphomas Treatment

A
  • Rapidly fatal if untreated but is curable
  • Treated aggressively at diagnosis - chemo + Rituximab
56
Q

Mantle Cell Lymphomas Treatment

A

Treated according to patient tolerance

57
Q

MALT Lymphomas Treatment

A

Triple Antibiotic therapy if localized disease

58
Q

Indolent lymphomas

A
  • Follicular Lymphoma
  • Marginal Zone Lymphoma
59
Q

Aggressive Lymphomas

2 listed

A
  • Burkitt Lymphoma
  • DLBCL

Hematology (26 decks)

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