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Heme and Lymph Exam 1 > Chapter 13: Common Forms of Lymphoid Leukemia and Lymphoma > Flashcards

Chapter 13: Common Forms of Lymphoid Leukemia and Lymphoma Flashcards

1
Q

There is an association with smoking and which neoplastic proliferation of white cells?

A

Acute myeloid leukemia

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2
Q

Among lymphoid cells, potentially oncogenic mutations occur most frequently where and during what?

A

Germinal center B cells during attempted antibody diversification

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3
Q

What is CD11c expressed on?

A
  • Granulocytes, monocytes, and macrophages
  • Also expressed by hairy cell leukemias
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4
Q

How does the spread of Hodgkin lymphoma differ from that of NHL’s?

A
  • Hodgkin spreads in an orderly fashion
  • Most forms of NHL spread widely early in their course and in less predictable fashion
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5
Q

Any child presenting with very low platelet counts has what until proven otherwise?

A

Acute lymphoblastic leukemia (ALL)

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6
Q

B-ALL is most often associated with what translocation?

A

t(12;21)

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7
Q

What is the cell of origin for B-ALL?

A

Bone marrow precursor B cell

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8
Q

90% of ALLs have numerical or structural chromosomal changes; most common is what?

A

Hyperploidy (>50 chromosomes)

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9
Q

Which immunostaining marker and B-cell markers are positive in B-ALLs?

A
  • (+) for TdT
  • Express CD10, CD19, and CD20
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10
Q

A majority of T-ALLs have gain of function mutation in what gene?

A

NOTCH1

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11
Q

Which CD markers are expressed by the cells of T-ALLs?

A

CD2 to CD8

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12
Q

Although genetically and immunophenotypically distinct, what are the clinical features shared between ALL and AML?

A
  • Abrupt onset within days to weeks
  • Sx’s related to depression of marrow function, fever, infection, bleeding
  • Mass effects, including bone pain; generalized LAD, hepatosplenomegaly; testicular enlargement
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13
Q

CNS manifestations such as HA, vomiting, and nerve palsies from meningeal spread are more common in which neoplasia of WBC’s?

A

ALL

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14
Q

What are 3 factors associated with a worse prognosis of ALL?

A
  • Age <2 y/o
  • Presentation in adolescence or young adults
  • Peripheral blasts >100,000
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15
Q

What are 5 features of ALL which would be associated with a favorable prognosis?

A
  • Age btw 2-10 y/o
  • Low WBC count
  • Hyperdiploidy
  • Trisomy of chromosomes 4, 7, and 10
  • Presence of t(12;22)
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16
Q

What are the most common genetic anomalies associated with CLL/SLL?

A

Trisomy 12, deletions of 11q, 13q, and 17p

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17
Q

Which distinct CD markers are expressed by the cells of CLL/SLL?

A

CD19 and CD20, as well as CD5*** and CD23

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18
Q

When present, which finding in the lymph nodes is pathognomonic for CLL/SLL?

A

Proliferation centers = large lymphocytes gathered in loos aggregates that contain mitotically active cells

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19
Q

What is the absolute lymphocyte count of peripheral blood in CLL that distinguishes it from SLL?

A

>5000 mm3

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20
Q

Uncommon, nucleated B cells known as prolymphocytes are sometimes seen in the lymph nodes assoc. w/ what neoplasm?

A

CLL/SLL

21
Q

What is a characteristic finding on peripheral smear in CLL?

A

Smudge cells

22
Q

How does the leukocyte count vary between CLL and SLL?

A
  • CLL can have leukocyte counts in excess of 200,000 m3
  • SLL can present have leukopenia with marrow involvement
23
Q

Most common age group and salient clinical features of CLL/SLL; minority of patients develop what?

A
  • Older adults w/ bone marrow, LN, spleen, and liver disease
  • Autoimmune hemolysis and thrombocytopenia in a minority
24
Q

Which finding due to disruption of normal immune function is common in CLL/SLL?

A

HYPOgammaglobulinemia which ↑ susceptibility to infections

25
Q

The presence of which mutated gene is associated with worse outcomes in CLL/SLL?

A

NOTCH1

26
Q

Development of a rapidly enlarging mass within a LN or spleen in pt with CLL/SLL is suggestive of what?

A

Richter syndrome –> prolymphocyte transformation to diffuse large B cell lymphoma = ominous event; survival <1 year

27
Q

Follicular lymphoma arises from where; what is the hallmark translocation?

A
  • Arise from germinal center B cells
  • Hallmark = t(14;18) –> BCL2-IgH fusion gene
28
Q

Follicular lymphoma most often occurs in what age group and which sex?

A

Middle aged adults; M=F

29
Q

Which 4 features of follicular lymphoma distinguish it from reactive follicular hyperplasia?

A
  • Disruption of normal LN architecture
  • Lack of tingible body macrophages in germinal centers
  • Bcl2 expression in follicles
  • Monoclonality
30
Q

The neoplastic cells of follicular lymphoma express which markers?

A
  • CD19, CD20, CD10; surface Ig, and BCL6
  • BCL2 is expressed in 90%
31
Q

Typical presentation of follicular lymphoma?

A
  • Older adult w/ painless generalized LAD and marrow involement
  • Extranodal involvement of GI tract, CNS, or testis = UNCOMMON
32
Q

What is the typical course and survival like for follicular lymphoma?

A
  • Incurable, usually follows indolent waxing and waning course
  • Median survival = 7-9 years
33
Q

How likely is histologic transformation of follicular lymphoma?

A

30-50% transform into DLBCL

34
Q

What is the peripheral blood and bone marrow involvement like in follicular lymphoma; which other sites are commonly involved?

A
  • Peripheral blood may have lymphocytosis (<20,000) in 10%
  • Bone marrow involvement characterized by paratrabecular lymphoid aggregates
  • Splenic WHITE pulp and hepatic portal triads frequently involved
35
Q

What are the salient clinical features of diffuse large B-cell lymphoma; which ages?

A
  • All ages, but most common in older adults (median age = 60)
  • Often appears as rapidly growing mass at nodal or extranodal site
36
Q

Diffuse large B-cell lymphoma is associated with what genetic anomalies?

A
  • Dysregulation of BCL6 (essential for normal germinal center formation)
  • Small % have t(14;18) - BCL2
37
Q

Which sites are commonly and uncommonly involved with diffuse large B-cell lymphoma?

A
  • Waldeyer ring, the oropharyngeal lymphoid tissue including tonsils and adenoids = frequently involved
  • 1’ or 2’ involvement of spleen and liver = large destructive masses
  • Extranodal involvement of GI tract, skin, bone, and brain
  • BM involvement = uncommon
38
Q

How does the subtype of DLBCL, Primary Effusion Lymphoma, typically present and the tumor cells are always infected with what?

A
  • Malignant pleural or ascitic effusion, mostly in pt’s with advaned HIV or older adults
  • In all cases the tumor cells infected w/ KSHV/HHV-8
39
Q

The immunodeficiency-associated large B-cell lymphoma is associated with what infection?

A

B cells are infected with EBV

40
Q

Burkitt lymphoma is associated with what translocation; which genes on each chromosome?

A

t(8;14) = MYC-IgH

41
Q

Unlike other tumors of germinal B-cell origin, Burkitt lymphome virtually always fails to express what?

A

The anti-apoptotic protein BCL2

42
Q

What is the significance of increased MYC levels associated with Burkitt Lymphoma?

A

↑ genes required for aerobic glycolysis (Warburg effect) allowing cells to biosynthesize all the building blocks needed for cell growth and division

43
Q

Both endemic and sporadic Burkitt Lymphomas are found mainly in whom and most tumors are found where?

A

Mainly in children or young adults; most arising at extranodal sites

44
Q

Which extra-nodal sites do the endemic vs. sporadic form of Burkitt Lymphoma have predilection for?

A
  • Endemic often presents as mass involving the mandible and has unusual predilection for abdominal viscera (kidneys, ovaries, adrenals)
  • Sporadic most often appears as mass involving ileocecum and peritoneum
45
Q

Essentially all Burkitt Lymphomas of which subtype are latently infected with EBV; small percentage of which other subtypes?

A

- ALL endemic (African) form

  • Also 25% of HIV-associated tumors + 15-20% of sporadic cases
46
Q

The tumor cells of Burkitt Lymphoma will express what?

A

Mature B cell markers = IgM, CD19, CD20, CD10, and BCL6

47
Q

Which pattern/morphology is seen in tissues with Burkitt Lymphoma?

A
  • Tumors w/ high mitotic index and numerous apoptotic cells
  • Phagocytes w/ abundant clear cytoplasm, creating characteristic “starry sky” pattern
48
Q

Typical behavior of Burkitt Lymphoma; response to treatment?

A
  • Very aggressive but responds well to intensive chemotherapy
  • Most children/young adults can be cured
  • Outcome is more guarded in older adults

Heme and Lymph Exam 1 (14 decks)

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