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Heme/Lymph Exam 1 (EH) > 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?

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
The presence of which mutated gene is associated with worse outcomes in CLL/SLL?
***NOTCH1***
26
Development of a rapidly enlarging mass within a LN or spleen in pt with CLL/SLL is suggestive of what?
**Richter syndrome** --\> **prolymphocyte** transformation to **diffuse large B cell lymphoma** = **ominous event**; survival \<1 year
27
Follicular lymphoma arises from where; what is the hallmark translocation?
- Arise from **germinal center B cells** - **Hallmark** = **t(14;18)** --\> ***BCL2-IgH*** fusion gene
28
Follicular lymphoma most often occurs in what age group and which sex?
**Middle aged adults**; **M=F**
29
Which 4 features of follicular lymphoma distinguish it from reactive follicular hyperplasia?
- **Disruption** of normal LN **architecture** - **Lack** of **tingible body macrophages** in **germinal centers** - **Bcl2 expression** in **follicles** - **Monoclonality**
30
The neoplastic cells of follicular lymphoma express which markers?
- **CD19, CD20, CD10**; surface **Ig**, and **BCL6** - **BCL2** is expressed in **90%**
31
Typical presentation of follicular lymphoma?
- **Older adult** w/ **painless generalized LAD** and **marrow** involement - **Extranodal involvement** of GI tract, CNS, or testis = **UNCOMMON**
32
What is the typical course and survival like for follicular lymphoma?
- **Incurable**, usually follows **indolent waxing** and **waning course** - **Median survival** = **7-9 years**
33
How likely is histologic transformation of follicular lymphoma?
**30-50%** transform into **DLBCL**
34
What is the peripheral blood and bone marrow involvement like in follicular lymphoma; which other sites are commonly involved?
- **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
What are the salient clinical features of diffuse large B-cell lymphoma; which ages?
- **All ages**, but **most common** in **older adults (median age = 60)** - Often appears as **rapidly growing mass** at **nodal** or **extranodal site**
36
Diffuse large B-cell lymphoma is associated with what genetic anomalies?
- **Dysregulation** of **BCL6** (essential for normal germinal center formation) - Small % have **t(14;18) - BCL2**
37
Which sites are commonly and uncommonly involved with diffuse large B-cell lymphoma?
- **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
How does the subtype of DLBCL, Primary Effusion Lymphoma, typically present and the tumor cells are always infected with what?
- **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
The immunodeficiency-associated large B-cell lymphoma is associated with what infection?
**B cells** are infected with **EBV**
40
Burkitt lymphoma is associated with what translocation; which genes on each chromosome?
**t(8;14) =** ***MYC-IgH***
41
Unlike other tumors of germinal B-cell origin, Burkitt lymphome virtually always fails to express what?
The **anti-apoptotic** protein **BCL2**
42
What is the significance of increased MYC levels associated with Burkitt Lymphoma?
↑ genes required for **aerobic glycolysis (Warburg effect**) allowing cells to **biosynthesize** all the building blocks needed for **cell growth** and **division**
43
Both endemic and sporadic Burkitt Lymphomas are found mainly in whom and most tumors are found where?
**Mainly** in **children** or **young adults**; most arising at **extranodal sites**
44
Which extra-nodal sites do the endemic vs. sporadic form of Burkitt Lymphoma have predilection for?
- **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
Essentially all Burkitt Lymphomas of which subtype are latently infected with EBV; small percentage of which other subtypes?
**- _ALL_ endemic (African) form** - Also **25%** of **HIV-associated** tumors + **15-20%** of **sporadic cases**
46
The tumor cells of Burkitt Lymphoma will express what?
**Mature B cell markers** = **IgM, CD19, CD20, CD10**, and **BCL6**
47
Which pattern/morphology is seen in tissues with Burkitt Lymphoma?
- Tumors w/ **high mitotic index** and **numerous apoptotic cells** - Phagocytes w/ abundant **clear cytoplasm**, creating characteristic **"starry sky" pattern**
48
Typical behavior of Burkitt Lymphoma; response to treatment?
- **Very aggressive** but responds **well** to **intensive chemotherapy** - **Most** children/young adults can be **cured** - Outcome is more **guarded** in **older adults**

Heme/Lymph Exam 1 (EH) (18 decks)

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