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Hematopathology WHO > Mature B cell Neoplasms Part I > Flashcards

Mature B cell Neoplasms Part I Flashcards

1
Q

Define CLL

A
  • Small mature B cells that coexpress CD5 and CD23
  • Must have a monoclonal B cell count of greater than or equal to 5 x 109/L
    • With the characteristic morphology and phenotype of CLL in the peripheral blood
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2
Q

What is the definition of monoclonal B cell lymphocytosis?

A
  • Clonal CLL count < 5 x 109/L
  • Without lymphadenopathy, organomegaly or any other extramedullary disease
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3
Q

What is the definition of SLL?

A
  • SLL and CLL are essentially the same disease…
  • Use the term SLL for the following situation:
    • circulating CLL count in peripheral blood <5 x 109/L
    • there is documented nodal, splenic, or other extramedullary involement
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4
Q

What is the epidemiology of CLL/SLL?

A
  • Most common leukemia of adults in Western countries
  • median patient age is 70 years old
  • more common in males (2:1)
  • Accounts for 7% of non-Hodgkin Lymphomas
  • Rare in Asian countries
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5
Q

What tissues are involved by CLL/SLL?

A
  • Blood and bone marrow
  • Secondary lymphoid tissues like the spleen, lymph nodes, Waldeyer’s ring
  • Extranodal involvement is not common but can occur
    • Skin
    • GI tract
    • CNS
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6
Q

How is CLL/SLL often diagnosed?

A
  • Generally it is diagnosed by routine blood work, occasionally by splenomegaly or lymphadenopathy
  • Other manifestations:
    • Autoimmune hemolytic anemia
    • Immune thrombocytopenia
    • Erythroblastopenia
    • Pulmonary infections
    • Severe allergic reactions to insect bites
    • IgM paraprotein (usually)
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7
Q

What is the morphology of CLL/SLL within the lymph nodes?

A
  • Diffuse architectural effacement by proliferation of small B lymphocytes
  • Variably scattered paler proliferation centres (pseudofollicles)
  • Sometimes have retained variably nodular appearance
  • Predominant cells in diffuse areas:
    • small lymphocytes with scant cytoplasm
    • usually round nucleus with clumped chromatin and occasional nucleolus
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8
Q

What are some of the variations in morphologies that can be seen in CLL/SLL?

A
  • moderate nuclear irregularity can lead to the differential diagnosis of Mantle Cell Lymphoma
  • Plasmacytoid differentiation can also be seen
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9
Q

What are the proliferation centres composed of in CLL/SLL?

A
  • continuum of small lymphocytes, prolymphocytes and paraimmunoblasts
  • Prolymphocytes:
    • small to medium sized
    • relatively clumped chromatin and small nucleoli
  • Paraimmunoblasts:
    • larger with round to oval nuclei, dispersed chromatin
    • central eosinophilic nucleolus
    • slightly basophilic cytoplasm
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10
Q

What can be seen with large proliferation centres in CLL/SLL?

A
  • these centres are usually large (broader than a 20x field)
  • they can also be confluent
  • Such cases are usually associated with increased proliferation
    • Also deletion of 17p13.1
    • Trisomy 12
    • More aggressive course
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11
Q

What is the morphology of CLL/SLL in the spleen?

A
  • white pulp involvement is usually very prominent
  • red pulp is also involved
  • proliferation centres may be seen but are less prominent than in lymph nodes
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12
Q

What is the morphology of CLL/SLL in the peripheral blood?

A
  • Small lymphocytes with clumped chromatin and scant cytoplasm
  • Smudge or basket cells are typically seen
  • Prolymphocytes can be seen:
    • larger with more dispersed chromatin
    • irregular nuclear contours
    • more abundant cytoplasm
    • consistently <15% of the cells
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13
Q

How is atypical CLL defined?

A
  • more prolymphocytes than normal CLL (>15%) but there is still <55%
  • Usually these cases are associated with the following:
    • Trisomy 12
    • Strong positivity for:
      • Surface immunoglobulin
      • CD20
      • FMC7
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14
Q

If you have >55% prolymphocytes what is your diagnosis?

A

B-cell prolymphocytic leukemia

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15
Q

What is the morphology of CLL/SLL in the bone marrow?

A
  • can have interstitial, nodular, combined or diffuse involvement
  • Diffuse involvement usually is seen in more advanced disease
  • Paratrabecular aggregates are not typical
  • Proliferation centres can be seen
  • Most cases have >30% CLL cells in the marrow aspirate
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16
Q

Immunophenotype of CLL/SLL

A
  • Positive for:
    • CD19, CD20, CD22 and CD79b
    • Dim surface IgM/IgD
    • CD5 and CD43
    • Strong positive: CD23 and CD200
  • ​​​Negative for:
    • CD10 and FMC7 (may be weakly expressed)
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17
Q

What is the atypical immunophenotype that may be seen with CLL/SLL ?

A
  • Negative:
    • CD5 or CD23
  • Positive
    • FMC7
    • Strong surface immunoglobulin
    • or CD79b
  • In these cases, especially CD5 negative, important to exclude:
    • Splenic Marginal Zone Lymphoma
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18
Q

What can the staining be in tissue sections of CLL/SLL?

A
  • cytoplasmic immunoglobulin can stain
  • CD20 and CD23 stronger positivity in the proliferation centres vs. the diffuse areas
  • LEF1
    • almost exclusively positive in CLL/SLL
    • not positive in normal B lymphocytes or other lymphomas
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19
Q

Can Cyclin D1 be positive in CLL/SLL?

A
  • YES!
  • some positive cells can be seen in proliferation centres in ~30% of cases
    • These cells are SOX11 negative
    • No chromosomal translocations are seen affecting the CCND1 gene

Note: MYC and NOTCH can also be seen in proliferation centres without gene alterations

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20
Q

What is the postulated normal counterpart to the CLL/SLL cell?

A
  • An antigen-experienced mature CD5+ B cell with mutated or unmutated IGHV genes
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21
Q

What are some of the most common cytogenetic abnormalities identified in CLL/SLL?

A
  • No specific genetic markers
  • Most common alterations identified include:
    • deletions in 13q14.3 (~50% of cases)
    • Trisomy 12 or partial trisomy 12q13 (~20% cases)
    • Deletion 11q22-23, 17p13.1 (TP53), or 6q21
  • Chromosomal translocations are uncommon in CLL
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22
Q

What is the inheritability of CLL/SLL?

A
  • Familial predisposition can be seen in 5-10% of patients
  • Risk of developing CLL is 2-7x higher in first degree relatives with CLL
  • Also at risk of other lymphoid neoplasms
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23
Q

What is the name of the clinical staging system used for CLL/SLL?

A

Rai and Binet are used to define disease extent and prognosis

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24
Q

True or False:

Patients who have CLL/SLL and have the mutated IGHV genes have a better prognosis that those who do not have the mutated gene.

A

True

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25
Q

The expression of what three markers in CLL/SLL has an adverese prognosis in the disease?

A

ZAP70

CD38

CD49d

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26
Q

How do the three epigenetic subtypes of CLL/SLL affect the prognosis of the disease?

A
  • Naive-like cases: have the worst prognosis
  • Memory-like cases: have the best prognosis
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27
Q

How do deletions of 11q and particularly deletion in 17p affect the clinical outcome of CLL/SLL?

A

Worse clinical outcome

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28
Q

What is the clinical outcome of deltion 13q14 in CLL/SLL?

A

Associated with a more favorable clinical course.

  • HOWEVER: CLL with a high proportion of cells with isolated 13q deletion do NOT do well.
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29
Q

What gene abnormalities predict a lack of response to Fludarabine-containing regimes in CLL/SLL?

A
  • TP53 abnormalities:
    • Deletion of 17p13.1
    • TP53 mutations
  • These mutations must be checked before starting any type of therapy in these patients.
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30
Q

Additional adverse predictive factors for CLL/SLL

A
  • Complex karyotype: poor outcome
  • rapid lymphocyte doubling in the blood (<12 months)
  • elevated serum markers of rapid cell turnover:
    • thymidine kinase
    • beta-2 microglobulin
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31
Q

What other mutations in CLL have been associated with a poor outcome?

A
  • TP53
  • ATM
  • NOTCH1
  • SF3B1
  • BIRC3
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32
Q

What features of CLL/SLL are suggestive of clinical progression or histologically more aggressive lesions?

A
  • Increase in size of proliferation centres of the lymph nodes
    • Higher proliferation rate with confluent centres
      • Broader than a 20x field
      • Ki67 >40% or >2.4 mitosis in the proliferation centres *
  • Prolymphocytoid Transformation: more prolymphocytes in the blood

Note: ** this needs more data to be supported

Note: these cases have an outcome intermediate between Richter transformation (DLBCL) and traditional CLL

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33
Q

Does CLL ever transform into B-cell prolymphocytic leukemia?

A

No

By definition these are two separate entities and CLL does NOT transform into this.

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34
Q

What diseases can CLL transform into and what is the prognosis?

A
  • DLBCL (~2-8% of cases)
  • Classic Hodgkin Lymphoma (<1%)
  • RICHTER Syndrome: cases of DLBCL that are clonally related to the previous CLL
    • Express the same immunoglobulin gene rearrangement and are IGHV-unmutated
  • Clonally unrelated CLL and DLBCL usually occur in IGVH mutated CLL
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35
Q

What is the prognosis of Richter transformation DLBCL in patients with CLL?

A

Median survival is <1 year

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36
Q

What is the prognosis of CLL with an unrelated, de novo DLBCL?

A

Prognosis is that of de novo DLBCL

37
Q

What mutations is DLBCL transformation from CLL associated?

A

TP53 mutation

NOTCH1 mutation

CDKN2A deletions

MYC translocation

38
Q

In what situation does Hodgkin Lymphoma develop in patients with CLL/SLL?

A
  • Mutated CLL
  • Hodgkin lymphoma cases are EBV positive
  • Hodgkin cases are unrelated to the CLL clone
39
Q

What is required to make the diagnosis of Hodgkin Lymphoma in the setting of CLL?

A
  • Classic Reed-Sternberg cells in an appropriate background of cells

Note: the presence of EBV positive or sometimes negative RS cells only is not enough to call Hodgkin lymphoma.

40
Q

What is the definition of a monoclonal B-cell lymphocytosis (MBL)?

A
  • Monoclonal B-cell count <5 x 109/L in the peripheral blood
  • NO associated lymphadenopathy, organomegaly, other extramedullary involvement
  • No other features of lymphoproliferative disorder

IMP: remember that many small B-cell lymphomas and leukemias have low-level peripheral blood involvement

41
Q

What are the three categories of Monoclonal B cell lymphocytosis?

A
  • CLL-type
  • Atypical CLL-type
  • non-CLL type
42
Q

Discuss the features of MBL with CLL-type

A
  • Most common type of MBL (~75% of cases)
  • Characterized by coexpression:
    • CD19, CD20 (dim), CD5 and CD23
    • B cells show light chain restriction or >25% lack surface immunoglobulin
  • More than 1 clone may exist
  • Frequency increases with age and all CLL cases develop from MBL but not all MBL proceed to CLL
43
Q

CLL-type MBL can be further classified how?

A
  • Further divided by the size of the monoclonal population
    • Low-count (<0.5 x109/L)
    • High-count (>0.5x109/L)
  • Low-count is biologically different and does not seem to progress to frank CLL
  • High-count has biological features similar to CLL and progresses to frank leukemia at an annual rate of 1-2% per year

​

44
Q

What is the definition of MBL with an atypical CLL phenotype?

A
  • Express:
    • CD19, CD20(bright), CD5
    • CD23 can be negative!
    • moderate to bright surface Ig

CRITICAL: to exclude a Mantle Cell Lymphoma or other B-cell lymphoma in these cases

45
Q

What is the definition of MBL with a non-CLL phenotype?

A
  • CD5 (-) or CD5 (dim)
  • CD19 and CD20 (+)
  • Moderate to right surface immunoglobulin expression

Note:

  • some clones may be transient or self-limited
  • upt to 17% may eventually develop splenomegaly, suggesting a relationship to Splenic Marginal Zone lymphoma
46
Q

What is the definition of B-cell Prolymphocytic Leukemia?

A
  • B-cell neoplasm composed of >55% of prolymphocytes within the peripheral blood
  • Involves other areas including:
    • bone marrow
    • spleen
47
Q

Why are cases of CLL with increased prolymphocytes and lymphoid proliferations with relatively similar morphology but a t(11;14)(q13;q32) [IGH/CCND1] translocation OR SOX11 excluded from the definition of B-cell prolymphocytic leukemia?

A

They are essentially Mantle Cell Lymphoma with leukemic expression.

48
Q

What is the incidence and median age of presentation of B-PLL?

A
  • represents about 1% of lymphocytic leukemias
  • usually seen in patients >60 years old
49
Q

Where are the leukemic cells localized in B-PLL?

A
  • peripheral blood
  • bone marrow
  • spleen
50
Q

What are the clinical feature of B-PLL?

A
  • B symptoms
  • Massive splenomegaly
  • Absent or minimal peripheral lymphadenopathy
  • Rapidly increasing lymphocyte count
    • >100 x 109/L
  • Anemia and thrombocytopenia are seen in 50% of cases
51
Q

What are the microscopic findings in the peripheral blood in B-PLL?

A

Peripheral Blood

  • Majority of circulating cells are prolymphocytes
    • >55%, but usually >90%
  • Medium sized lymphocytes
    • 2x the size of a normal lymphocyte
  • Round nucleus, moderately condensed nuclear chromatin, and prominent central nucleolus
    • some cases the nucleus can be indented
  • Relatively small amount of faintly basophilic cytoplasm
52
Q

What are the microscopic findings in the bone marrow in B-PLL?

A
  • interstitial or nodular intertrabecular infiltrate
  • lymphoid cells are similar to those seen in the peripheral blood
53
Q

What are the microscopic findings in the spleen in B-PLL?

A
  • expanded white pulp nodules
  • red pulp infiltrate
  • intermediate to large cells
  • abundant cytoplasm, irregular round nuclei and prominent eosinophilic nucleolus
54
Q

What are the microscopic findings in the lymph nodes in B-PLL?

A
  • diffuse or vaguely nodular infiltrate by cells similar in appearance to those in the spleen
  • proliferation centres (pseudo-follicles) are not seen
55
Q

True or False

The diagnosis of B-PLL can be made easiy on morphologic grounds?

A

False

  • No specific markers for B-PLL
  • Diagnosis of B-PLL cannot be made without excluding the following diseases:
    • pleomorphic mantle cell lymphoma
    • splenic marginal zone lymphoma
    • CLL with an increased number of prolymphocytes
56
Q

What is the immunophenotype of B-PLL?

A
  • strongly express surface IgM/IgD
  • B cell antigens (strongly expressed)
    • CD19, CD20, CD22, CD79a, CD79b, and FMC7
    • CD5: positive in only 20-30% of cases
    • CD23: positive in only 10-20% of cases
    • CD200: weakly positive or negative
57
Q

Does B-PLL show CD38 and ZAP70 expression?

A
  • 50% of cases show expression of the antigens
  • ZAP70 expression does NOT correlate with IGHV mutation status
58
Q

What is the postulated normal counterpart for B-PLL?

A
  • A mature B cell of unknown type
59
Q

Is t(11;14)(q13;q32) identified in B-PLL?

A
  • NO
  • These are considered to be leukemic forms of Mantle Cell Lymphoma
60
Q

What cytogenetic abnormalities have been identified in B-PLL?

A
  • Complex karyotypes are common
  • Deletions in 17p13 are detected in 50% of cases
    • these are associated with TP53 mutations
    • This likely underlies the progressive course of B-PLL and relative treatment resistance
  • Deletions of 13q14 are seen in 27% of cases
  • Trisomy 12 is uncommon
  • Aberrations of MYC including gains, amplifications and translocations are reported
  • IMP: B-PLL has a transcriptional profile that is different from CLL
61
Q

What is the prognosis of B-PLL and are there any predictive factors ?

A
  • Median survival: 30-50 months
    • B-PLL responds poorly to therapies for CLL
  • NO Correlation between survival and the following:
    • ZAP70 expression
    • CD38 positivity
    • Deletion 17p
    • IGHV mutation status
  • splenectomy may improve symptoms
62
Q

What is the definition of splenic marginal zone lymphoma?

A
  • Small, B-cell neoplasm
  • Lymphocytes replace the splenic white pulp germinal centres
    • efface the follicle mantle
    • merge with the peripheral (marginal) zone of larger cells
    • scattered transformed blasts are present
    • small and large cells infiltrate the red pulp
63
Q

What other sites can and often does splenic marginal zone lymphoma involve?

A
  • splenic hilar lymph nodes
  • bone marrow
  • lymphoma cells are often found in the peripheral blood as villous lymphocytes
  • Note:
    • the liver can often be involved as well
    • the peripheral lymph nodes are NOT involved
64
Q

What is the epidemiology of splenic marginal zone lymphoma (SMZL)?

A
  • represents <2% of all lymphoid neoplasms
  • but it may be a significant portion of otherwise unclassifiable CLL cases that are CD5 negative
  • usually seen in patients >50 years old
  • women and men are equally affected
65
Q

What is the clinical presentation of SMZL?

A
  • Splenomegaly
  • often have autoimmune thrombocytopenia and anemia
  • bone marrow is frequently involved
  • 1/3 of patients have a small paraprotein
    • hyperviscosity and hypergammaglobulinemia are rare
  • IMP: there is an association between SMZL and Hepatits C virus in southern Europe
66
Q

What are the microscopic findings of SMZL in the spleen?

A
  • splenic white pulp has a central zone of small, round lymphocytes often replacing any germinal centers
  • the normal mantle is effaced as well
  • merge with a peripheral zone of medium-sized lymphocytes
    • cells have dispersed chromatin and abundant pale cytoplasm
      • resemble marginal zone cells
      • transformed blasts are also present
67
Q

What does the red pulp look like in SMZL?

A
  • red pulp is always infiltrated by nodules of larger cells and sheets of small lymphocytes
    • often invade the sinuses
  • Epithelioid histiocytes can be present within the lymphoid aggregates
68
Q

True or False:

Plasmacytic differentiation is seen in SMZL?

A

TRUE

  • plasmacytic differentiation can occur
  • RARELY clusters of plasma cells can be seen in the centre of the white pulp nodules
69
Q

What are the microscopic findings of SMZL in the splenic hilar lymph nodes?

A
  • sinuses are dilated and lymphoma cells surround the germinal centres
  • unlike in the spleen
    • the lymphoma cells and more marginal-zone like cells are more intimately intermixed
    • there is no formation of the so-called marginal zone
70
Q

What are the microscopic findings of SMZL in the bone marrow?

A
  • nodular interstitial infiltrate, cytologically similar to that seen in the lymph nodes
  • sometimes neoplastic cells surround reactive follicles
  • intrasinusoidal lymphocytes are more evident after staining with CD20
  • IMP:
    • peripheral blood cells have short polar villi
    • sometimes can look plasmacytoid
71
Q

What is the differential diagnosis of SMZL?

A
  • CLL
  • Hairy cell leukemia
  • Mantle Cell Lymphoma
  • Follicular lymphoma
  • Lymphoplasmacytic lymphoma
72
Q

What are important things to remember when thinking of the differential for SMZL?

A

​

  • Many small B cell lymphomas can have slightly larger cells with pale cytoplasm when they involve the splenic marginal zone (mimic SMZL)
  • Nodular infiltration pattern in bone marrow excludes hairy cell leukemia
73
Q

What is the immunophenotype of SMZL?

A
  • express surface IgM and usually IgD
  • Positive
    • CD20, CD79a
  • Negative
    • CD5 and CD10
    • CD23 and CD43
    • Annexin 1
    • CD103 and cyclin D1
  • Ki67 usually in a targetoid pattern
    • positive in GC and marginal zone
74
Q

The absence of staining of cyclin D1 and LEF1 in SMZL is helpful in excluding which two entities?

A
  • Cyclin D1: Mantle Cell Lymphoma
  • LEF1: CLL
75
Q

The absence of staining of Annexin 1, CD10 and BCL6 in SMZL helps exclude which entities?

A
  • Annexin 1: Hairy Cell Leukemia
  • CD10 and BCL6: Follicular Lymphoma

IMP: a group of CD5+ SMZL cases has been described and are characterized by a higher lymphocytosis and diffuse bone marrow infiltration.

76
Q

What is the postulated normal counterpart of SMZL?

A

Marginal Zone B cell

may or may not demonstrate evidence of antigen exposure

77
Q

What are the key cytogenetic abnormalities seen in SMZL?

A
  • SMZL lacks recurrent chromosomal translocations which makes identification of such useful in diagnosing other B cell lymphomas that may mimic SMZL
  • Small number show t(2;7)(p12;q21) translocation
    • activates the CDK6 gene
  • 30% SMZL have heterozygous deletion of 7q
    • rarely found in other lymphomas
  • Gain of 3q is seen in a good subset
78
Q

What two genes are frequently mutated in SMZL that give a hint as to the pathogenesis of the disease?

A

NOTCH2

  • one of the most frequently mutated (10-25%)
  • also seen in other B cell lymphomas

KLF2

  • somatically mutated in 10-40% of cases
  • also seen in other B cell lymphomas

Both mutations are seen with cases with deletion 7q

The genes are physiologically inovlved in proliferation and commitment of mature B cells to the marginal zone, points to homing to the spleen compartments and marginal zone differentiation

79
Q

Presence of MYD88 mutations point to which entity instead of SMZL?

A

Lymphoplasmacytic lymphoma

Note: although rarely SMZL can have this mutation.

80
Q

Presence of t(14;18)(q32;q21) translocation points to which entity rather than SMZL and what are the genes?

A
  • Follicular Lymphoma
  • affects BCL2
81
Q

Presence of t(11;14)(q13;q32) translocation points to which entity rather than SMZL and what are the genes?

A
  • Mantle Cell Lymphoma
  • CCND1
82
Q

Presence of t(11;18)(q21;q21), t(14;18)(q32;q21), and t(1;14)(p22;q32) translocation points to which entity rather than SMZL and what are the genes?

A
  • Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)
  • Genes (respective to each translocation)
    • t(11;18)(q21;q21): BIRC3-MALT1
    • t(14;18)(q32;q21): IGH/MALT1
    • t(1;14)(p22;q32): IGH-BCL10
83
Q

What is the prognosis of SMZL?

A
  • clinical course is indolent with a 10 year survival of 67-95%
  • Repsponse to chemotherapy often used for other B cell lymphomas is poor
  • Patients often respond (hematologically) to splenectomy and Rituximab with long-term survival
  • Transformation to Large B cell lymphoma occurs in 10-15% of cases
84
Q

Does SMZL associated with Hepatitis C virus infection respond to antiviral treatment?

A
  • YES
  • Treatment with Interferon gamma with or without Ribavirin has been shown to be effective in treating SMZL
85
Q

What are some adverse prognostic factors in SMZL?

A
  • large tumor mass
  • poor general health status
  • NOTCH2, KLF2, and in particular TP53 mutations

Note: a clinical scoring system has been proposed that looks at the following to risk stratefy:

  • Hgb concentration
  • Platelet count
  • Lactate dehydrogenase
  • Extrahilar lymphadenopathy
86
Q

What is the definition of Hairy Cell Leukemia?

A
  • Indolent
  • small mature B lymphocytes
  • oval nuclei and abundant cytoplasm with hairy projections
  • involves the peripheral blood and diffusely infiltrates the bone marrow and splenic red pulp
87
Q

What is the epidemiology of Hairy Cell Leukemia?

A
  • Rare disease, accounts for only 2% of all lymphoid leukemias
    • Annual incidence rate: 0.32 per 100,000
  • Median age of presentation is 58 years of age
    • Rare in patients in their 20s
    • Very uncommon diagnosis in children
  • More common in males (4:1)
  • More common in whites than in blacks
88
Q

What is the disease defining molecular alteration in Hairy Cell Leukemia?

A
  • BRAF V600E
  • This mutation leads to a constituitive activation of MAPK
  • This mutation is present in 100% of cases
89
Q

What are the sites of involvement for Hairy Cell Leukemia?

A
  • Lesions are predominantly found in the bone marrow and spleen
  • Typically a small number of tumor cells can be found circulating in the peripheral blood
  • Tumor infiltrates can be noted in the liver and the lymph nodes (rarely)
    • Occasionally in the skin
  • Very rarely patients can present with abdominal lymphadenopathy

Hematopathology WHO (29 decks)

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