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Boards.CP.Heme > SLL/CLL,MCL,FL > Flashcards

SLL/CLL,MCL,FL Flashcards

-General -SLL/CLL -Mantle Cell -Follicular

1
Q

Follicular Lymphoma Variant - Isolated GI:

  • prognosis
  • location
A

Isolated GI FL:

  • Excellent prognosis
  • Duodenum
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2
Q

Mantle Cell Lymphoma:

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

A

Cyclin-D1 (bcl-1) amplification

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3
Q

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

-Age prediliction

A
  • Low-Grade - Older Adults

- High-Grade - Children/Young Adults

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4
Q

SLL/CLL - Chromosomal status by FISH:

-Good prognosis

A
  • Normal karyotype

- del(13q)

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5
Q

Follicular Lymphoma - Architecture:

  • Proliferations
  • Capsule
  • Follicles (4)
A
  • 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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6
Q

Follicular Lymphoma - Immunohistochemistry:

-Negative for (3)

A
  • CD5
  • CD43
  • CD11c
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7
Q

Follicular Lymphoma - Presentation

A

Isolated lymphadenopathy withOUT constitutional symptoms

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8
Q

Follicular Lymphoma - Tumor Cell Morphology:

A

2 Cell Types:

  • Centrocytes (small cleaved cells)
  • Centroblasts (large noncleaved cells)
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9
Q

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

A
  • Cytopenia
  • M protein
  • B symptoms (fever, night sweats, weight loss)
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10
Q

Which has the most aggressive clinical course?

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

Mantle Cell Lymphoma

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

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11
Q

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

  • Low-Grade (1 and 2)
  • High-Grade (3A and 3B)
A 
Grade 1: 0-5
Grade 2: 6-15
Grade 3: >15
-3A: Some residual centrocytes
-3B: No centrocytes
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12
Q

Mantle Cell Lymphoma:

-Poor Prognostic findings

A
  • Mitotic rate: >10 mitosis/hpf

- Ki-67: >40%

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13
Q

SLL/CLL - Immunophenotype:

-Positive in 50% of cases (2)

A
  • CD38

- ZAP-70

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14
Q

SLL/CLL - Immunophenotype:

-Positive for (5)*

A
  • CD5
  • CD23
  • CD43
  • CD79a
  • CD11c (dim/variable)

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

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15
Q

Centrocytes vs. Centroblasts:

-Large noncleaved cells

A

Centroblasts (Blasts are Big and Bare)

*Centrocytes (Cytes are Small and Cleaved)

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16
Q

Mantle Cell Lymphoma - Variants (4)

A
  • Blastoid*
  • Pleomorphic*
  • Small Cell
  • Marginal Zonelike

*More Aggressive

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17
Q

SLL/CLL - Chromosomal status by FISH:

-Poor prognosis

A
  • del(11q)

- del(17p)

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18
Q

Follicular Lymphoma Variant - Isolated Cutaneous:

  • prognosis
  • IHC
  • Cytogenetics
A

Isolated Cutaneous FL:

  • Excellent prognosis
  • Lacks CD10 and bcl-2 expression (bcl-6+)
  • Lacks BCL2 rearrangement
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19
Q

SLL/CLL - MC genetic abnormality by FISH

A

Deletion of 13q14 (>50%)

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20
Q

SLL transformation into Large Cell Lymphoma is called what?

A

Richter syndrome

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21
Q

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

A

CD10

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22
Q

Mantle Cell Lymphoma Tumor Cell Morphology:

  • Size
  • Nuclear contour
  • Nucleoli
  • Mitosis
  • Mixture of…
A
  • Small to Medium sized lymphs
  • Irregular nuclear contours
  • Small, subtle nucleoli
  • Frequent Mitosis
  • Mixture of histiocytes and Hyalinized Vessels
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23
Q

Follicular Lymphoma - Molecular and Cytogenetics

  • Translocation
  • Rearrangement
  • Most sensitive assay
A
  • t(14;18)
  • BCL2 on 18 with J region of IgH on 14
  • FISH most sensitive assay
24
Q

Follicular Lymphoma - Immunohistochemistry:

-Positive for (9)

A
  • CD10
  • CD23
  • FMC-7
  • bcl-6

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

25
Q

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

A

True

26
Q

What helps differentiate SLL from Mantle Cell lymphoma?

A
  • CD23+ in SLL (Neg. in MCL)
  • dim CD20+ in SLL (Bright in MCL)
  • “cloudy sky” appearance in SLL
27
Q

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

A
  • DLBCL
  • Follicular lymphoma
  • CLL/SLL
  • Mantle Cell lymphoma
28
Q

Follicular Lymphoma-Bone Marrow Finding

A

Focal Paratrabecular agregates

29
Q

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

  • Which are often disseminated at presentation?
  • Which are often localized?
A
  • Low-Grade (disseminated)

- High-Grade (localized)

30
Q

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

A

True

31
Q

SLL/CLL - Clinical Presentation:

  • Age
  • Findings (3)
  • Autoimmunity/Immunodeficiency
  • M protein
A
  • 65 years (mean)
  • Adenopathy, Splenomegaly, Peripheral and BM involvement
  • Autoimmunity common (+DAT in 30%)
  • Immunodeficiency common (hypogammaglobulinemia in 30-50%)
  • M protein occasionally present
32
Q

What must the lymphocyte count exceed to call CLL?

A

5 x 10^9/L (5,000/uL)

monoclonal B cell lymphocytosis under this number

33
Q

Mantle Cell Lymphoma Immunophenotype:

-Negative for (4)

A
  • CD10
  • CD11c
  • CD23
  • CD99
34
Q

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

A
  • CD19
  • CD20*
  • CD22
  • sIg*
  • bcl-2

*CD20/sIg are dim in CLL/SLL and Bright in Mantle/Follicular

35
Q

SLL/CLL - Immunophenotype:

-Negative for (3)

A
  • FMC-7
  • CD10
  • bcl-6
36
Q

SLL/CLL - Tumor Cell Morphology:

  • Size
  • Nuclear contours
  • Chromatin
A

SLL Tumor Cells - Small lymphocytes

  • Rounded nuclear contours
  • Clumped chromatin
37
Q

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

A

GI tract (Stomach-MC)

38
Q

SLL/CLL - MC cytogenetic abnormality

A

Trisomy 12 (20%)

39
Q

Mantle Cell Lymphoma:

-Molecular and Cytogenetics

A

t(11;14)
-rearrangement of JH region of IgH (14) to the CCND1 (11)

*most have additional abnormalities, MC affecting chr13

40
Q

SLL - Morphology

-Architecture

A

SLL Architecture:

  • Diffuse Nodal effacement
  • Proliferation centers (pseudofollicular growth centers); many prolymphocytes
41
Q

What is the most common presentation in B-cell neoplasms?

-Other presentations (3)

A

Lymphadenopathy

Other:

  • Lymphocytosis
  • Extranodal tumor
  • Secondary Effects
42
Q

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

A

True

*its the MC encountered in B lineage lymphoma

43
Q

Percentage of Prolymphocytes:

  • CLL
  • CLL/PLL
  • true PLL
A
  • <11% (CLL)
  • 11-55% (CLL/PLL)
  • > 55% (PLL)
44
Q

Follicular Lymphoma:

-Poor prognostic Factors (5)

A
  • Older age
  • Serum LD
  • BM involvement
  • B symptoms
  • Anemia
45
Q

Mantle Cell Lymphoma Morphology:

-Architecture

A
  • Diffuse or “Vaguely Nodular” lymph node effacemet

- NO proliferation centers (No prolymphocytes)

46
Q

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

A

Overexpression of bcl-2 protein

-Antiapoptotic properties

47
Q

Which B-cell neoplasm displays the strongest genetic influence?

A

SLL/CLL

-Risk in 1st degree relatives 5X

48
Q

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

A
  • Immunodeficiency

- Autoimmunity

49
Q

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

A

40%

50
Q

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

A

Female predominance in:

  • Primary Mediastinal Lymphoma
  • Follicular Lymphoma
  • MALT Lymphoma
51
Q

Mantle Cell Lymphoma - Presentation

-Common sites

A

Adenopathy

  • Waldeyer Ring
  • GI tract (lymphomatous polyposis)
52
Q

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

A

-EDTA

NOT seen in Heparin smears

53
Q

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

A

90%

54
Q

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

A

CD21

CD23

55
Q

What is the most common B-cell Leukemia?

A

CLL

56
Q

Mantle Cell Lymphoma Immunophenotype:

-Positive for (4)

A
  • CD5
  • CD43
  • FMC-7
  • bcl-1 (cyclin D1, prad 1)

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

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