DLBCL

Question 1

Hans Method - DLBCL subtypes:

-Step 3

Answer 1

MUM1

• Positive, then Non-GBC (ABC)
• Negative, then GBC

Question 2

What is true about late (>5 years) PTLD?

Answer 2

• EBV Negative

- More Aggressive

Question 3

Posttransplant Lymphoproliferative DIsorder (PTLD):

• When do they most often occur?
• What are the “heralded” by?

Answer 3

• <1 year post-transplant

- Heralded by elevated EBV-DNA

Question 4

Lymphomatoid Granulomatosis most commonly affects which organs? (4)
-What is it associated with?

Answer 4

-Respiratory
-Brain
-Kidney
-Liver
Associated with EBV and Immunodeficiency

Question 5

DLBCL clinical presentation.

Answer 5

• Rapidly enlarging lymph node or extranodal site
• Localized at presentation

*BM involvement uncommon (10%)

Question 6

ALK+ DLBCL:

• Neoplastic cells
• Nodal or Extranodal
• HIV or EBV association

Answer 6

• Immunoblastic/Plasmablastic that express ALK
• Nodal
• NOT ass. w/ HIV or EBV

Question 7

What are the most common lymphomas in HIV? (5)

Answer 7

• Burkitt Lymphoma
• DLBCL (CNS)
• Primary Effusion Lymphoma
• Plasmablastic Lymphoma
• Hodgkin Lymphoma

Question 8

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

-Small lymphocyte IHC (+2;-3)

Answer 8

Positive:
-Mixture of CD4+ and CD8+
Negative:
-CD57
-CD21
-CD23
*NO T-cell rosettes or small B cells

Question 9

Primary Mediastinal (Thymic) Large B-cell Lymphoma:

• Age
• Morphology

Answer 9

• Adult Females (F:M;2:1)

- Sclerosing lymphoma with large B cells entrapped within bands of sclerosis

Question 10

Primary Mediastinal (Thymic) Large B-cell Lymphoma:
-IHC (+5;-3)

Answer 10

Positive:
-CD19
-CD20
-CD30
-CD45
-CD79a
Negative:
-CD5
-CD10
-Surface Igs

Question 11

Hans Method - DLBCL subtypes:

-Step 1

Answer 11

CD10

• Positive (>30%), then GCB
• Negative, step 2

Question 12

Ki67 expression in DLBCL.

Answer 12

60-99%

Question 13

Which type of DLBCL, Germinal Center (GCB) or Activated B-cell (ABC; nonGCB), has a better response to treatment?

Answer 13

Germinal Center (GCB) type

Question 14

TCRBCL Bone Marrow involvement pattern.

Answer 14

Paratrabecular lymphoid aggregates

Question 15

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

• Age
• Morphology

Answer 15

T-Cell/Histiocyte Rich DLBCL (TCRBCL):

• 40 y/o (mean; kids-elderly)
• Diffuse proliferation of small lymphocytes and histiocytes with scattered large B cells

Question 16

Primary Mediastinal (Thymic) Large B-cell Lymphoma:
-Molecular/Cytogenetics

Answer 16

Alterations in the MAL gene
-Gains in 9p (location of JAK2)

*NO rearrangement of BCL2 or BCL6

Question 17

Rearrangement of BCL6 is more common in what type of DLBCL?

Answer 17

Non-Germinal Center (ABC) type

Question 18

CD5 expressing DLBCL must be distinguished from what?

Answer 18

Blastoid MCL (bcl-1+)

Question 19

What are the most common Primary Immunodeficiencies (PID) with Lymphoproliferative disorders (LPD)? (5)

Answer 19

• Ataxia Telangiectasia
• Wiskott Aldrich syndrome
• Common Variable Immunodeficiency
• X-linked Lymphoproliferative disorder (Duncan disease)
• Nijmegan Chromosomal Breakage Syndrome

Question 20

What gene rearrangements are present in 20-30% of DLBCL?

Answer 20

BCL2 and BCL6

Question 21

Hans Method - DLBCL subtypes:

-Step 2

Answer 21

BCL6

• Negative, then Non-GBC (ABC)
• Positive, step 3

Question 22

What is Primary Effusion Lymphoma (PEL) associated with?

Answer 22

HHV8 and HIV

Question 23

DLBCL is what percentage of non-Hodgkin lymphomas?

Answer 23

30%

Question 24

DLBCL IHC:

-Positive for (5)

Answer 24

• CD19
• CD20
• CD22
• CD45
• bcl-2

Question 25

DLBCL IHC: | -Variable for (3)

Answer 25

- CD5 - CD10 - bcl-6

Question 26

What rearrangement is more common in GCB-type DLBCL?

Answer 26

BCL2 | -t(14;18)

Question 27

Primary CNS DLBCL: | -Molecular/Cytogenetics

Answer 27

Most cases have BCL6 rearrangement -Overexpress bcl-6 *BCL2 rearrangement is RARE

Question 28

Intravascular Large B cell lymphoma is AKA (3). - Symptoms due to what? - Nodal or Extranodal

Answer 28

-Angioednotheliomatosis -Angiotropic lymphoma -Intravascular lymphomatosis Small vessel obstruction by large B cells -LN involvement is RARE

Question 29

Where is the BCL6 gene located? - What is it commonly rearranged with? - Non-GCB (ABC) type or GCB type

Answer 29

3(q27) - t(3;14) - Non-GCB (ABC)

Question 30

What is HHV8/HIV associated with? (3)

Answer 30

- PEL - Castleman - Kaposi

Question 31

T-Cell/Histiocyte Rich DLBCL (TCRBCL): | -Large B cell IHC (+3;-3)

Answer 31

``` Positive: -pan B-cell -bcl-6 -EMA (+/-) Negative: -CD15 -CD30 -EBV ```

Question 32

Where is the BCL2 gene located? | -Non-CGB (ABC) type or GCB type?

Answer 32

18(q21) | -GCB type

Question 33

Lymphomatoid Granulomatosis (LG) morphology and cell types.

Answer 33

- Vasculitis pattern (Large Bs invade vessel wall) - Large number of reactive T-cells, plasma cells, and histiocytes - Granulomas UNcommon

Question 34

PTLD Risk Factors: - Renal/BMT recipients - Heart-Lung/Liver-Bowel - Age - Pretransplant EBV status - Degree of Immunosuppression

Answer 34

PTLD Risk Factors: - Renal/BMT- Lower Risk - Heart-Lung/Liver-Bowel - Higher Risk - Age - Kids at Higher Risk - Pretransplant EBV status - Negative - Degree of Immunosuppression - Most suppressed; Highest risk

Question 35

Plasmablastic DLBCL: - Neoplastic cells - Positive IHC (5) - Negative IHC (3) - Nodal or Extranodal - HIV or EBV association

Answer 35

``` -Immunoblastic/Plasmablastic Positive IHC: -CD38 -CD138 -MUM1 (IRF4) -cytoplasmic Ig -EBV Negative IHC: (contrast to plasmacytoma) -CD20 -CD45 -CD56 Extranodal -mucosa of oral cavity Ass. w/ HIV and EBV ```

Question 36

What are the most common Lymphoproliferative disorders (LPD) associated with Primary Immunodeficiencies (PID)? (3)

Answer 36

- DLBCL - Lymphomatoid Granulomatosis - T-Cell neoplasm

Question 37

Primary Effusion Lymphoma IHC: | -Positive for (6)

Answer 37

- HHV8 - CD30 - CD45 - CD38 - CD138 - EMA

Question 38

Primary CNS DLBCL: - Age - Radiographic - May present or recur as - Histology

Answer 38

Primary CNS DLBCL: - 60 y/0 - Supratentorial mass (looks like GBM) - Intraocular lymphoma - Perivascular cuffs (express pan B cell antigens)