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AP/CP Board Review Hemepath > B cell neoplasms: CLL, Mantle cell, & Follicular > Flashcards

B cell neoplasms: CLL, Mantle cell, & Follicular Flashcards

1
Q

What can be the clinical

presentation of B cell neoplasms ?

A
  • lymphadenopathy
  • peripheral blood lymphocytosis
  • cytopenia
  • M protein
  • B symptoms
    • fever, night sweats, and loss of >10% body weight
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2
Q

What are the common sites for

extranodal B cell lymphomas?

A
  • GI tract
    • stomach is most common
  • Skin
  • Bone
  • CNS
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3
Q

What B cell neoplasms can present

as leukemia ?

A
  • CLL
  • Prolymphocytic leukemia
  • Hairy cell leukemia
  • B-ALL
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4
Q

B cell neoplasms have various tendencies for marrow involvement. Which neoplasms

involve the marrow in a paratrabecular pattern?

A
  • Follicular lymphoma
    • 1/3 grade discordant with lymph node and bone marrow, can be prognostically important
  • sometimes Marginal zone lymphoma but not frequently
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5
Q

Which B cell neoplasms involve the marrow in a nodular pattern ?

A
  • Mantle cell
    • 20% involve the marrow
  • Marginal zone
    • 5% involve marrow
    • 80% involve the peripheral blood
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6
Q

Which lymphoma involves the bone marrow in

an interstitial pattern?

A
  • lymphoplasmacytic lymphoma
    • 10% involvement of bone marrow
    • 10% involve peripheral blood
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7
Q

Which lymphomas can involve the bone marrow

in a diffuse pattern ?

A
  • DLBCL
    • 15% involve the bone marrow
  • Burkitt lymphoma
    • 5% involve marrow
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8
Q

What are some of the risk factors

for developing B cell neoplasms?

A
  • immunodeficiency
  • autoimmunity
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9
Q

What is the most common B cell

leukemia in adults in the Western hemisphere ?

A
  • CLL/SLL
    • CLL demonstrates the strongest genetic influence
      • familial clustering in 5% of cases
      • 5X increased risk in first degree relatives of affected individuals
    • incidence increases with age, median age of 65 years
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10
Q

What is the clinical presentation of CLL/SLL ?

A
  • generalized lymphadenopathy, splenomegaly, peripheral blood lymphocytosis
  • KNOW- autoimmunity and immunodeficiency are common
  • cytopenias
    • caused by autoimmunity rather than by marrow replacement
  • positive DAT
    • full blown autoimmune hemolytic anemia
  • M protein- occasional
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11
Q

What is the morphology of SLL ?

A
  • diffuse nodal effacement by small, mature appearing lymphocytes
    • rounded nuclear contours
    • coarse chromatin
    • scant cytoplasm
  • Proliferation centers (pseudofollicles)
    • pale nodules
    • filled with prolymphocytes and paraimmunoblasts
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12
Q

What is thought to be a histologic subtype

with aggressive clinical behavior

for SLL?

A
  • when the proliferation centers are expanded and highly active
    • Ki67 > 40%
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13
Q

What is the morphology of

CLL ?

A
  • lymphocytosis composed of small, mature appearing lymphocytes with scant cytoplasm
  • coarsely clumped chromatin
  • smudge cells
    • this is an EDTA artifact
    • not seen on Heparin smears
    • can reduce by replacing albumin
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14
Q

What is the definition of

CLL by the WHO ?

A
  • absolute lymphocyte count > 5 x 10 ^9/L
    • or 5,000/uL
    • can be the sole criterion
    • but if you have tissue infiltration by CLL then just call CLL
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15
Q

What is the definition of

monoclonal B cell lymphocytosis (MBL) ?

A
  • monoclonal B cell population
  • immunophenotypically identical to CLL
  • BUT
    • no tissue involvement
    • < 5 x 10 ^9/L (5,000/uL)
  • MBL can be found in 3.5% of the population normally when over 40 years old
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16
Q

What is the morphology of

Prolymphocytes in CLL ?

A
  • prolymphocytes are characterized by an increased volume of cytoplasm, open chromatin, and central prominent nucleoli
  • should comprise 10% or less of the population
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17
Q

What is the definition of CLL

with increased prolymphocytes ?

A
  • 11-55% prolymphocytes in addition to the normal CLL population

Note: prolymphocyte counts of >15 x 10^9/L appear to have poor clinical outcome

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18
Q

What is the definition of prolymphocytic leukemia?

A
  • > 55% prolymphocytes
  • rarely evolves from CLL
    • often is de novo
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19
Q

What is the pattern of involvement

of CLL in the bone marrow ?

A
  • nodular
    • good prognosis
  • diffuse
    • poor prognosis
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20
Q

What is the immunophenotype of

CLL/SLL ?

A
  • Positive
    • CD19, CD20 (dim), CD22
    • CD5, CD23
    • surface Ig (dim)
    • CD43, CD79a
    • CD11c (dim and variable)
  • LEF1
    • expression is highly specific for CLL/SLL
  • Negative :
    • FMC7, CD10, BCL6, and BCL1
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21
Q

In CLL, what does expression of

CD38 and ZAP70 imply?

A
  • implies an unmutated IgVH status and an unfavorable diagnosis
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22
Q

What are the common cytogenetic

findings in in CLL/SLL ?

A
  • 30% of cases have complex abnormalities
  • deletion of 13q14 (50%) **
  • trisomy 12 (20%)
  • del (11q)
  • del(14q)
  • del(17p)

Richter Syndrome/transformation to a large B cell lymphoma occurs in 3-15% of cases

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23
Q

What is the single most important

prognostic factor in CLL/SLL ?

A
  • mutation status of the IgH variable region
    • CLL with unmutated IgVH resembles pre-GCB cells and are likely to have poor prognosis
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24
Q

What are other indicators of

worsened survival in CLL?

A
  • B symptoms
  • peripheral lymphocyte doubling time less than 1 year
  • higher initial lymphocyte count (esp >30,000)
25
Q

What is correlated with ZAP70 or CD38 +

in CLL ?

A
  • expression correlates with atypical morphology
  • unmutated IgVH and worsened prognosis

Note: for designation of CD38+, over 30% of neoplastic cells should express CD38

26
Q

How do the cytogenetics of CLL affect

the prognosis of patients ?

A
  • 11q and 17p deletions
    • shortest survival with these findings
  • isolated del 13q14
    • stable course
  • isolated trisomy 12
    • impact unclear
    • generally considered adverse
  • t(14;19)
    • translocation associated with bcl3 expression
    • atypical morphology, trisomy 12
    • aggressive disease
27
Q

How is CLL treated?

A
  • watchful waiting
  • Fludarabine: purine analog
    • with chemo and rituximab, or alone
  • Ibrutanib
    • used in CLL with del17p
    • also in relapsed refractory cases
28
Q

What are some general facts about

Mantle Cell Lymphoma ?

A
  • aggressive disease with median survival of 3-4 yrs
  • occurs in adults, usually men
  • often occurs in Waldeyer’s ring and the GI tract
    • Lymphomatoid polyposis
      • endoscopic involvement in 60% of patients
29
Q

What is the morphology of Mantle Cell Lymphoma?

A
  • diffuse or vaguely nodular effacement of the lymph node architecture
  • small to medium sized lymphocytes
    • irregular contours
    • small subtle nucleolus
    • look like boulders
  • Germinal centers without mantle zones
    • pink histiocytes
    • hyalinized blood vessels
30
Q

What are the variants of Mantle Cell Lymphoma ?

A
  • blastoid
  • pleomorphic
  • small cell
  • marginal zone like
  • peripheralized (nodal MCL with circulating cells)
  • leukemic nonnodal mantle cell lymphoma
31
Q

What is the morphology of a blastoid and pleomorphic variants

of MCL ?

A

* Both are considred more aggressive variants

Blastoid Variant

  • large cells with open chromatin
  • high mitotic rate

Pleomorphic Variant

  • mixture of cell sizes
  • many large cells with nucleoli
32
Q

What is the main differential for

MCL ?

A
  • Lymphoblastic leukemia/lymphoma
    • will lack BCL1 and CD5
    • will express CD99 and Tdt
33
Q

How can mantle cell neoplasia in situ be

identified vs. reactive follicles ?

A
  • cyclin D1 expression is seen in the mantle zones
34
Q

What is the characteristic immunophenotype for

Mantle Cell Lymphoma ?

A
  • Positive
    • CD19, CD20 (bright), CD22
    • FMC7, CD5, CD43
    • surface Ig (bright)
    • bcl 1 (cyclin D1)
    • more likely to be lambda restricted compared to kappa
  • Negative
    • CD23
    • CD11c
    • CD10
35
Q

What is the utility of SOX11 in Mantle Cell Lymphoma ?

A
  • Sox11 is a transcription factor
  • increased sensitivity and specificity
  • often used in cyclin-D1 negative cases
36
Q

What are the cytogenetics of Mantle cell lymphoma?

A
  • t(11;14)
    • results in a translocation of IgH (14q32) gene of the CCND1 (11q13)
    • results in cyclin D1 amplification identified by FISH
37
Q

What are the key prognostic things for Mantle cell lymphoma ?

A
  • single most important factor is proliferative activity
    • mitosis > 50 per mm^2 - correlate with worse prognosis
    • Ki67 > 30% correlates with adverse prognosis

Note: blastoid and plemorphic morpholgy are thought to be adverese

38
Q

What is the common clinical presentation of

Follicular lymphoma ?

A
  • uncommon to be found in extranodal sites
  • isolated lymphadenopathy without constitutional symptoms
  • bone marrow is involved in ~30% of cases
    • paratrabecular growth
39
Q

What is the morphology of Follicular lymphoma ?

A
  • nodular lymphoid proliferation that typically overruns the lymph node capsule (uniform in size cells)
  • back to back fused follicles with attenuated mantles
    • loss of polarity of GC
    • absence of tingible body macrophages
    • diminished mitosis
  • Note: see diffuse areas and sclerosis in mesenteric or retroperitoneal LN
40
Q

What are the 2 cell types present in

Follicular Lymphoma ?

A
  • 2 cells types are present in varying proportions
    • centrocytes
    • large noncleaved cells (centroblasts)
41
Q

How is grading of Follicular Lymphoma performed

and what is considered Grade 1 ?

A
  • based on the proportion of large non-cleaved cells (centroblasts)
    • evaluate a 40x field of 10 randomly selected neoplastic follicles
    • do not specifically pick the worse areas
  • Grade 1:
    • 0-5/40x

Note: Grade 1 and 2 are assigned as low-grade

42
Q

How are Grade 2 and 3 assigned to a Follicular Lymphoma ?

A
  • Grade 2:
    • 6-15/ 40x HPF
  • Grade 3:
    • 3A: >15 /40x HPF
    • 3B: no centrocytes are visible in 40x field
43
Q

What is considered diffuse growthin in Follicular Lymphoma ?

A
  • an area where follicular architecture is lost
    • lacks FDC by CD21 or CD23 IHC
  • Grade 3 Diffuse areas have a diagnosis of DLBCL
    • background FL also noted
44
Q

What is the morphology of Follicular Lymphoma cells

in peripheral blood ?

A
  • buttock cell –> very indented nucleus
45
Q

What is the definition of in situ Follicular Neoplasia?

A
  • germinal center looks architecturally reactive
  • cytological features of follicular lymphoma
    • monomorphous, centrocyte rich
46
Q

What is the clinical presentation and important

characteristics of Primary cutaneous FL ?

A
  • low rate of LN lymph node involvement and an excellent overall prognosis
  • IHC
    • variable CD10, lack of bcl2 expression
    • bcl6 is positive
  • IF FL in skin expresses
    • CD10 and or BCL2
    • suspect systemic involvement by FL
47
Q

What is characteristic of the floral variant

of Follicular Lymphoma ?

A
  • mantle zone B cells penetrate into the neoplastic follicles
    • create irregular shapes
  • can highlight the morphology using FDC markers- CD21
  • often a Grade 3 lymphoma
48
Q

What is characteristic of the duodenal type

of Follicular Lymphoma ?

A
  • rare
  • presents as multiple polyps in the 2nd portion of the duodenum (most common site)
  • low rate of lymph node dissemination
  • excellent prognosis if surgically removed even
49
Q

What is important to know about testicular FL ?

A
  • High grade follicular lymphoma
    • especially in children
    • lacks the bcl2 translocation
  • Good prognosis
50
Q

What is important to know about

Pediatric Follicular lymphoma ?

A
  • often presents with localized cervical lymphadenopathy
  • lacks bcl2 expression
    • lacks bcl2 rearrangement
    • usually Grade 3
  • Excellent prognosis
51
Q

What is the pattern of

bone marrow involvement by

Follicular Lymphoma ?

A
  • focal paratrabecular aggregates
  • this pattern is only seen with FL and occasionally TCRBCL
    • Note: sometimes mantle cell can show this pattern
52
Q

Why is it important to evaluate the bone marrow

in Follicular Lymphoma ?

A
  • if there is a high grade lymphoma elsewhere, but lower grade in the bone marrow it imparts a better prognosis
    • worse prognosis if both are high grade
53
Q

What is the immunophenotype of

Follicular Lymphoma ?

A
  • Positive:
    • CD19, CD20, CD22
    • FMC7, CD10, surface Ig bright
    • bcl6 and bcl2

Note: CD10 and bcl2 can be lost in higher grade

  • Negative
    • CD5, CD43, CD11c
    • variable CD23

Note: CD21 and CD23 highlight FDCs, without staining supports a diffuse pattern

54
Q

What is the characteristic

cytogenetic alteration of

Follicular Lymphoma ?

A
  • t(14;18)
    • results in a BCL2 rearrangement with IGH
    • bcl2 protein overexpression
55
Q

What is the prognosis

of Follicular Lymphoma ?

A
  • median survival of 8-10 years
  • eventually progresses/transforms to high grade FL or DLBCL
56
Q

What factors have been associated with

progression in Follicular Lymphoma ?

A
  • age
  • stage
  • bone marrow involvement
  • B symptoms
  • performance status
  • serum LDH levels
  • anemia
57
Q

What is the clinical presentation of

Large B cell lymphoma with

IRF4 (MUM1) rearrangement ?

A
  • usually seen in children and young adults
  • involves Waldeyer’s ring
    • also seen in head and neck lymph nodes
  • Favorable prognosis
58
Q

What is the morphology and IHC

profile of Large B cell Lymphoma

with IRF4 (MUM1) rearrangement ?

A
  • diffuse
  • follicular and diffuse
  • or entirely follicular
  • usually Grade 3

Immunohistochemistry

  • (+) : MUM1 and bcl6
  • CD10 (+) in 65% of cases
59
Q

What is the genetic alteration

of Large B cell Lymphoma with

IRF4 rearrangment ?

A
  • t(6;14)
    • IGH-BCL2
  • usually IRF4 gene rearranged with BCL6

AP/CP Board Review Hemepath (12 decks)

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