Hematology Week 3: Lymphoma II Flashcards

(59 cards)

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
Mantle Cell Lymphoma Genetics
t(11;14)(q13;q32)
26
Mantle Cell Lymphoma Pathophysiology
* 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
Mantle Cell Lymphoma Genetic testing
* Karyotype * FISH
28
Mantle Cell Lymphoma Immunohistochemistry
29
Role of Cyclin D1 in cell cycle
passes G0 to S
30
Marginal Zone Lymphoma Follicle Histology
Arise from memory B cells in the marginal zone
31
Marginal Zone Lymphoma Morphology
32
MALT Lymphoma Associations 2 listed
33
Helicobacter pylori associations with lymphoma
34
MALT Lymphoma Clinical Course
35
MALT Lymphoma Immunophenotype 4 listed
* CD19+ * CD20+ * CD5- * CD10-
36
Burkitt Lymphoma Types 3 listed
* Endemic in Africa all EBV+ * Sporadic * Immunodeficiency-related (HIV)
37
Burkitt Lymphoma Histology
Identical histology in all types Starry-sky
38
Burkitt Lymphoma Immunophenotype 4 listed
* CD20+ * CD10+ * BCL6+ * BCL2-
39
Burkitt Lymphoma Genetics 3 listed
* t(8;14) in 80% * t(2;8) or t(8;22) in 20%
40
Burkitt Lymphoma Endemic type and lymphoma belt of Africa
41
Fastest growing tumor
Burkitt Lymphoma
42
Burkitt Lymphoma Molecular Pathogenesis
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
BurkitBurkitt Lymphoma genetic testing
Karyotype and FISH
44
Burkitt Lymphoma Pathophysiology
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
Clinical & Diagnostic utility of detection of specific chromosomal rearrangements 3 listed
46
The significance of distinction of subtypes of B-cell lymphoma
47
Antigen receptor gene rearrangements
48
Light and Heavy chain rearrangements
* 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
Clinical and diagnostic utility of IGH gene rearrangements 3 listed
PCR can identify clonal populations
50
Polyclonal vs monoclonal populations capillary electrophoresis
51
Question 1
Mantle Cell Lymphoma
52
Question 2 Mantle Cell Lymphoma
C * CD10+ * CD20+ * CD5+ * Cyclin D1+
53
Summary of Selected Mature B Cell Lymphomas
54
Indolent Lymphomas Treatment
* incurable in an advanced stage * Treat only symptomatic Disease * Rituximab + Chemotherapy
55
Aggressive Lymphomas Treatment
* Rapidly fatal if untreated but is curable * Treated aggressively at diagnosis - chemo + Rituximab
56
Mantle Cell Lymphomas Treatment
Treated according to patient tolerance
57
MALT Lymphomas Treatment
Triple Antibiotic therapy if localized disease
58
Indolent lymphomas
* Follicular Lymphoma * Marginal Zone Lymphoma
59
Aggressive Lymphomas 2 listed
* Burkitt Lymphoma * DLBCL