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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

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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?

Study These Flashcards
A
  • B symptoms
  • peripheral lymphocyte doubling time less than 1 year
  • higher initial lymphocyte count (esp >30,000)
25
What is correlated with ZAP70 or CD38 + in CLL ?
* expression correlates with atypical morphology * unmutated IgVH and worsened prognosis Note: for designation of CD38+, over 30% of neoplastic cells should express CD38
26
How do the cytogenetics of CLL affect the prognosis of patients ?
* **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
How is CLL treated?
* watchful waiting * Fludarabine: purine analog * with chemo and rituximab, or alone * Ibrutanib * used in CLL with del17p * also in relapsed refractory cases
28
What are some general facts about Mantle Cell Lymphoma ?
* 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
What is the morphology of Mantle Cell Lymphoma?
* 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
What are the variants of Mantle Cell Lymphoma ?
* blastoid * pleomorphic * small cell * marginal zone like * peripheralized (nodal MCL with circulating cells) * leukemic nonnodal mantle cell lymphoma
31
What is the morphology of a blastoid and pleomorphic variants of MCL ?
\* 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
What is the main differential for MCL ?
* Lymphoblastic leukemia/lymphoma * will lack BCL1 and CD5 * will express CD99 and Tdt
33
How can mantle cell neoplasia in situ be identified vs. reactive follicles ?
* cyclin D1 expression is seen in the mantle zones
34
What is the characteristic immunophenotype for Mantle Cell Lymphoma ?
* 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
What is the utility of SOX11 in Mantle Cell Lymphoma ?
* Sox11 is a transcription factor * increased sensitivity and specificity * often used in cyclin-D1 negative cases
36
What are the cytogenetics of Mantle cell lymphoma?
* t(11;14) * results in a translocation of IgH (14q32) gene of the CCND1 (11q13) * results in cyclin D1 amplification identified by FISH
37
What are the key prognostic things for Mantle cell lymphoma ?
* 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
What is the common clinical presentation of Follicular lymphoma ?
* uncommon to be found in extranodal sites * isolated lymphadenopathy without constitutional symptoms * bone marrow is involved in ~30% of cases * paratrabecular growth
39
What is the morphology of Follicular lymphoma ?
* 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
What are the 2 cell types present in Follicular Lymphoma ?
* 2 cells types are present in varying proportions * centrocytes * large noncleaved cells (centroblasts)
41
How is grading of Follicular Lymphoma performed and what is considered Grade 1 ?
* 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
How are Grade 2 and 3 assigned to a Follicular Lymphoma ?
* Grade 2: * 6-15/ 40x HPF * Grade 3: * 3A: \>15 /40x HPF * 3B: no centrocytes are visible in 40x field
43
What is considered diffuse growthin in Follicular Lymphoma ?
* 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
What is the morphology of Follicular Lymphoma cells in peripheral blood ?
* buttock cell --\> very indented nucleus
45
What is the definition of in situ Follicular Neoplasia?
* germinal center looks architecturally reactive * cytological features of follicular lymphoma * monomorphous, centrocyte rich
46
What is the clinical presentation and important characteristics of Primary cutaneous FL ?
* 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
What is characteristic of the floral variant of Follicular Lymphoma ?
* 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
What is characteristic of the duodenal type of Follicular Lymphoma ?
* 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
What is important to know about testicular FL ?
* High grade follicular lymphoma * especially in children * lacks the bcl2 translocation * Good prognosis
50
What is important to know about Pediatric Follicular lymphoma ?
* often presents with localized cervical lymphadenopathy * lacks bcl2 expression * lacks bcl2 rearrangement * usually Grade 3 * Excellent prognosis
51
What is the pattern of bone marrow involvement by Follicular Lymphoma ?
* focal paratrabecular aggregates * this pattern is only seen with FL and occasionally TCRBCL * Note: sometimes mantle cell can show this pattern
52
Why is it important to evaluate the bone marrow in Follicular Lymphoma ?
* 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
What is the immunophenotype of Follicular Lymphoma ?
* 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
What is the characteristic cytogenetic alteration of Follicular Lymphoma ?
* t(14;18) * results in a BCL2 rearrangement with IGH * bcl2 protein overexpression
55
What is the prognosis of Follicular Lymphoma ?
* median survival of 8-10 years * eventually progresses/transforms to high grade FL or DLBCL
56
What factors have been associated with progression in Follicular Lymphoma ?
* age * stage * bone marrow involvement * B symptoms * performance status * serum LDH levels * anemia
57
What is the clinical presentation of Large B cell lymphoma with IRF4 (MUM1) rearrangement ?
* usually seen in children and young adults * involves Waldeyer's ring * also seen in head and neck lymph nodes * Favorable prognosis
58
What is the morphology and IHC profile of Large B cell Lymphoma with IRF4 (MUM1) rearrangement ?
* diffuse * follicular and diffuse * or entirely follicular * usually Grade 3 Immunohistochemistry * (+) : MUM1 and bcl6 * CD10 (+) in 65% of cases
59
What is the genetic alteration of Large B cell Lymphoma with IRF4 rearrangment ?
* t(6;14) * IGH-BCL2 * usually IRF4 gene rearranged with BCL6

AP/CP Board Review Hemepath (12 decks)

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