General Principles of B cell neoplasia Flashcards

1
Q

B cell development and markers

A
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2
Q

Neoplasms arising from precursor B cells

A

B-acute lymphoblastic lymphoma

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3
Q

Pre-germinal center B cell neoplasms

A

Mantle cell lymphoma

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4
Q

Germinal center B cell neoplasms

A

Follicular lymphoma
Burkitt’s lymphoma
DLBCL, GCB type
Hodgkin’s lymphoma

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5
Q

Post-germinal center B cell neoplasms

A

Marginal zone lymphoma
Mucosal-associated lymphoid tissue lymphoma
CLL/SLL
DLBCL, Non-GCB type

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6
Q
A

Centroblast

Larger GC cell with open chromatin, high N:C ratio.

Centroblasts reside in the dark zone of the GC.

They express low levels of surface immunoglobulins and switch off BCL-2, making them susceptible to apoptosis.

They are CD10 positive, BCL6 positive, LMO2 positive, HGAL positive, and BCL2 negative.

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7
Q
A

Centrocyte

Smaller GC cells with angulated to twisted nuclei and a lower N:C ratio than centroblasts. Some just look like normal mature lymphocytes.

Reside in the light zone of the GC.

Express higher levels of surface immunoglobulins and have undergone somatic hypermutation and heavy chain class switching.

IRF4 (MUM1) downregulates BCL6 expression

BCL6 negative, IRF4/MUM1 positive.

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8
Q
A

Follicular dendritic cell

Larger cell with relatively round nucleus and a prominent central nucleolus. The chromatin is slightly finer and paler than surrounding lymphocytes in a germinal center.

Unlike many other DCs, follicular DCs are of mesenchymal origin, not hematopoietic origin.

Stain for CD21/CD23.

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9
Q
A

Marginal zone cells

Post-GC memory B cells.

Cytologically, MZ cells have small to medium size, pale-to-clear cytoplasm, irregular nuclei with dispersed chromatin, and inconspicuous nucleoli.

Reside in marginal zones of lymph nodes, spleen, and MALT. Marginal zones are often hard or impossible to see on H&E outside of the spleen.

Express pan-B antigens and surface IgM, sometimes low level IgD. CD5 and CD10 negative, BCL2 and HLA-DR positive.

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10
Q

What are the cells in the area highlighted by the yellow arrows?

A

Mantle cells

Present in all secondary follicles, immediately surrounding the germinal center.

Mantle cells are pre-germinal center and are thus IgD or IgM positive.

Wild-type mantle cells are TCL1 positive.

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11
Q

Plasma cell markers

A

CD138, BLIMP1, Cyclin D1

Loss of PAX5

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12
Q

Pan-B cell markers

A

CD19, CD79a

PAX5 and CD20 (except plasma cells)

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13
Q

WHO definition of CLL

A
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14
Q

WHO definition of SLL

A
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15
Q

WHO definition of monoclonal B-cell lymphocytosis

A
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16
Q

“CLL-type” monoclonal B-cell lymphocytosis

A
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17
Q

Low-count MBL
vs
High-count MBL

A
18
Q

Atypical or Non-CLL-type MBL

A
19
Q
A

Richter transformation

Transformation of an SLL into a DLBCL (or a Hodgkin lymphoma).

Occurs in 2-10% of patients with CLL/SLL.

The resultant DLBCL often loses CD5 expression and is typically of non-GCB type, but may occasionally be of GCB type.

20
Q

Richter transformation to Hodgkin lymphoma

A
21
Q

“Subtypes” of Mantle cell lymphoma presentation

A

Classic MCL: nodal or extra-nodal
Leukemic MCL: JUST circluating MCL cells

22
Q

The “lymphomatous polyposis” presentation of lymphoma in the GI tract is most commonly caused by. . .

A

Mantle cell lymphoma

23
Q

Blastoid MCL

A
24
Q

Leukemic MCL phenotype

A

Often CD5-, SOX11-

Can be tricky to diagnose, but you always can rely on the t(11;14) translocation to demonstrate Cyclin D1 is rearranged.

Leukemic MCL has a better overall survival than classical MCL.

25
Q

Reporting of follicular lymphoma pattern

A
26
Q

“Diffuse” pattern follicular lymphoma

A

Can be called ONLY if the lymphoma is low-rade (a grade 3A or 3B diffuse pattern would be DLBCL)

No follicular structures present, but follicular lymphoma by IHC and molecular features, with centrocytes +/- centroblasts.

27
Q

Follicular lymphoma grading in the WHO4R

A
28
Q

Follicular lymphoma grading in the WHO5

A
29
Q

Follicular lymphoma grading in the ICC

A
30
Q

How to decide between FL3A/cFL and FL3B/FLBCL

A
31
Q

I think it’s follicular lymphoma, but my BCL2 stain is negative.

What next?

A
32
Q

Juxtratrabecular
vs
Paratrabecular

A

Juxtatrabecular: Adjacent to, but not immediately abutting, the bony trabeculae. Seen in lymphoplasmacytic lymphoma.

Paratrabecular: Immediately abutting the bony trabeculae. Seen in follicular lymphoma.

33
Q

2nd most common mutation in LPL

A

CXCR4

30% of cases

34
Q

Other neoplasms with MYD88 L265P

A

IgM MGUS (but not IgG/IgA)

Nodal MZL
Splenic MZL
MALT lymphoma
CLL

Non-GC-type DLBCL
Over 50% of primary cutaneous DLBCL, leg type
DLBCL at immune privileged sites (testes, CNS)

35
Q

The variable LPL immunophenotype

A
36
Q

What are molecular features that can help distinguish LPL from IgM myeloma?

A

IgM myeloma usually has t(11;14) IgH::CyclinD1, and never has MYD88 L265P

37
Q

The many faces of nodal MZL

A
38
Q

Weirdly, hairy cell leukemia affects ____ the most

A

Monocytes

Hairy cell leukemia patients have basically no monocytes, so much so that if your patient DOES you should hesitate to diagnose hairy cell outright.

39
Q

The most specific markers for Hairy cell

A

BRAFV600E
CD103
Annexin A1

CD25+ and CD11c bright are good screening tools

40
Q

EBF1

A

Pioneer transcription factor that drives early chromatin remodeling to initiate the B cell program.

Recruits Brg1 and FUS via a prion-like C-terminal domain