Follicular Lymphoma Variant - Isolated GI: - prognosis - location

Isolated GI FL: - Excellent prognosis - Duodenum

Follicular Lymphoma - Architecture: - Proliferations - Capsule - Follicles (4)

- Nodular lymphoid proliferations (back-to-back, fused follicles with attenuated mantles) - Overruns the capsule Follicles Lack: - Polarity - Tingle body macrophages - Plasma cells - Mitosis (few)

Follicular Lymphoma Variant - Isolated Cutaneous: - prognosis - IHC - Cytogenetics

Isolated Cutaneous FL: - Excellent prognosis - Lacks CD10 and bcl-2 expression (bcl-6+) - Lacks BCL2 rearrangement

Mantle Cell Lymphoma Tumor Cell Morphology: - Size - Nuclear contour - Nucleoli - Mitosis - Mixture of...

- Small to Medium sized lymphs - Irregular nuclear contours - Small, subtle nucleoli - Frequent Mitosis - Mixture of histiocytes and Hyalinized Vessels

Follicular Lymphoma - Molecular and Cytogenetics - Translocation - Rearrangement - Most sensitive assay

- t(14;18) - BCL2 on 18 with J region of IgH on 14 - FISH most sensitive assay

SLL/CLL,MCL,FL Flashcards by Filip Garrett

Follicular Lymphoma Variant - Isolated GI:

prognosis
location

Isolated GI FL:

Excellent prognosis
Duodenum

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Mantle Cell Lymphoma:

-Rearrangement of JH region of IgH (14) to the CCND1 (11) results in what?

Cyclin-D1 (bcl-1) amplification

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Low-grade Vs. High-grade B cell neoplasms:

-Age prediliction

Low-Grade - Older Adults

- High-Grade - Children/Young Adults

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SLL/CLL - Chromosomal status by FISH:

-Good prognosis

Normal karyotype

- del(13q)

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Follicular Lymphoma - Architecture:

Proliferations
Capsule
Follicles (4)

Nodular lymphoid proliferations (back-to-back, fused follicles with attenuated mantles)
Overruns the capsule

Follicles Lack:

Polarity
Tingle body macrophages
Plasma cells
Mitosis (few)

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Follicular Lymphoma - Immunohistochemistry:

-Negative for (3)

CD5
CD43
CD11c

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Follicular Lymphoma - Presentation

Isolated lymphadenopathy withOUT constitutional symptoms

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Follicular Lymphoma - Tumor Cell Morphology:

2 Cell Types:

Centrocytes (small cleaved cells)
Centroblasts (large noncleaved cells)

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What are the “secondary effects” of B-cell Neoplasms?

Cytopenia
M protein
B symptoms (fever, night sweats, weight loss)

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Which has the most aggressive clinical course?

CLL/SLL
Follicular Lymphoma
Mantle Cell Lymphoma
Marginal Zone Lymphoma
Hairy Cell Leukemia

Mantle Cell Lymphoma

*Despite is benign appearance, it often presents with disseminated disease and survival of 3-4 years

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Follicular Lymphoma Grading - Based on number of centroblasts in 10 Random 40X fields:

Low-Grade (1 and 2)
High-Grade (3A and 3B)

Grade 1: 0-5
Grade 2: 6-15
Grade 3: >15
-3A: Some residual centrocytes
-3B: No centrocytes

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Mantle Cell Lymphoma:

-Poor Prognostic findings

Mitotic rate: >10 mitosis/hpf

- Ki-67: >40%

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SLL/CLL - Immunophenotype:

-Positive in 50% of cases (2)

CD38

- ZAP-70

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SLL/CLL - Immunophenotype:

-Positive for (5)*

CD5
CD23
CD43
CD79a
CD11c (dim/variable)

*CD19, CD20(dim), CD22, sIg(dim), bcl-2

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Centrocytes vs. Centroblasts:

-Large noncleaved cells

Centroblasts (Blasts are Big and Bare)

*Centrocytes (Cytes are Small and Cleaved)

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Mantle Cell Lymphoma - Variants (4)

Blastoid*
Pleomorphic*
Small Cell
Marginal Zonelike

*More Aggressive

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SLL/CLL - Chromosomal status by FISH:

-Poor prognosis

del(11q)

- del(17p)

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Follicular Lymphoma Variant - Isolated Cutaneous:

prognosis
IHC
Cytogenetics

Isolated Cutaneous FL:

Excellent prognosis
Lacks CD10 and bcl-2 expression (bcl-6+)
Lacks BCL2 rearrangement

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SLL/CLL - MC genetic abnormality by FISH

Deletion of 13q14 (>50%)

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SLL transformation into Large Cell Lymphoma is called what?

Richter syndrome

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Positivity for what marker becomes increasingly negative with higher grade in FL?

CD10

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Mantle Cell Lymphoma Tumor Cell Morphology:

Size
Nuclear contour
Nucleoli
Mitosis
Mixture of…

Small to Medium sized lymphs
Irregular nuclear contours
Small, subtle nucleoli
Frequent Mitosis
Mixture of histiocytes and Hyalinized Vessels

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Follicular Lymphoma - Molecular and Cytogenetics

Translocation
Rearrangement
Most sensitive assay

t(14;18)
BCL2 on 18 with J region of IgH on 14
FISH most sensitive assay

Follicular Lymphoma - Immunohistochemistry:

-Positive for (9)

CD10
CD23
FMC-7
bcl-6

*CD19, CD20(Bright), CD22, sIg(Bright), bcl-2

T/F: Pediatric Follicular Lymphoma is usually grade 3.

True

What helps differentiate SLL from Mantle Cell lymphoma?

- CD23+ in SLL (Neg. in MCL) - dim CD20+ in SLL (Bright in MCL) - "cloudy sky" appearance in SLL

Most Common types of B cell lymphomas. (4;Decreasing order)

- DLBCL - Follicular lymphoma - CLL/SLL - Mantle Cell lymphoma

Follicular Lymphoma-Bone Marrow Finding

Focal Paratrabecular agregates

Low-grade Vs. High-grade B cell neoplasms: - Which are often disseminated at presentation? - Which are often localized?

- Low-Grade (disseminated) | - High-Grade (localized)

T/F: Follicular Lymphoma may be "discordant," with LG in marrow and HG in lymph node.

True

SLL/CLL - Clinical Presentation: - Age - Findings (3) - Autoimmunity/Immunodeficiency - M protein

- 65 years (mean) - Adenopathy, Splenomegaly, Peripheral and BM involvement - Autoimmunity common (+DAT in 30%) - Immunodeficiency common (hypogammaglobulinemia in 30-50%) - M protein occasionally present

What must the lymphocyte count exceed to call CLL?

5 x 10^9/L (5,000/uL) | monoclonal B cell lymphocytosis under this number

Mantle Cell Lymphoma Immunophenotype: | -Negative for (4)

- CD10 - CD11c - CD23 - CD99

What Cell Markers are ALL positive in CLL/SLL, Mantle Cell lymphoma, and Follicular lymphoma? (5) *Intensity differences

- CD19 - CD20* - CD22 - sIg* - bcl-2 *CD20/sIg are dim in CLL/SLL and Bright in Mantle/Follicular

SLL/CLL - Immunophenotype: | -Negative for (3)

- FMC-7 - CD10 - bcl-6

SLL/CLL - Tumor Cell Morphology: - Size - Nuclear contours - Chromatin

SLL Tumor Cells - Small lymphocytes - Rounded nuclear contours - Clumped chromatin

Which organ is most commonly involved by a B-cell Extranodal tumor?

GI tract (Stomach-MC)

SLL/CLL - MC cytogenetic abnormality

Trisomy 12 (20%)

Mantle Cell Lymphoma: | -Molecular and Cytogenetics

t(11;14) -rearrangement of JH region of IgH (14) to the CCND1 (11) *most have additional abnormalities, MC affecting chr13

SLL - Morphology | -Architecture

SLL Architecture: - Diffuse Nodal effacement - Proliferation centers (pseudofollicular growth centers); many prolymphocytes

What is the most common presentation in B-cell neoplasms? | -Other presentations (3)

Lymphadenopathy Other: - Lymphocytosis - Extranodal tumor - Secondary Effects

T/F: The t(14;18) translocation is NOT unique to Follicular Lymphoma.

True *its the MC encountered in B lineage lymphoma

Percentage of Prolymphocytes: - CLL - CLL/PLL - true PLL

- <11% (CLL) - 11-55% (CLL/PLL) - >55% (PLL)

Follicular Lymphoma: | -Poor prognostic Factors (5)

- Older age - Serum LD - BM involvement - B symptoms - Anemia

Mantle Cell Lymphoma Morphology: | -Architecture

- Diffuse or "Vaguely Nodular" lymph node effacemet | - NO proliferation centers (No prolymphocytes)

Rearrangement of BCL2 on chr18 with the J region of IgH on chr14 results in what?

Overexpression of bcl-2 protein | -Antiapoptotic properties

Which B-cell neoplasm displays the strongest genetic influence?

SLL/CLL | -Risk in 1st degree relatives 5X

What are the only significant risk factors B-cell neoplasms?

- Immunodeficiency | - Autoimmunity

What % of B-cell neoplasms have BM involvement at presentation?

40%

Most B-cell neoplasms display male predominance, what are the Exceptions?

Female predominance in: - Primary Mediastinal Lymphoma - Follicular Lymphoma - MALT Lymphoma

Mantle Cell Lymphoma - Presentation | -Common sites

Adenopathy - Waldeyer Ring - GI tract (lymphomatous polyposis)

Which medium causes CLL "smudge cells"? (which one are they not seen)

-EDTA | NOT seen in Heparin smears

What percentage of lymphoid neoplasms are composed of mature B-cells?

90%

What markers do background follicular dendritic cells (FDC) express?

CD21 | CD23

What is the most common B-cell Leukemia?

CLL

Mantle Cell Lymphoma Immunophenotype: | -Positive for (4)

- CD5 - CD43 - FMC-7 - bcl-1 (cyclin D1, prad 1) *CD19, CD20(Bright), CD22, sIg(Bright), bcl-2

SLL/CLL,MCL,FL Flashcards

-General -SLL/CLL -Mantle Cell -Follicular