Unit 2: Lymphocytic Leukemias Flashcards

1
Q

Occurs when massive proliferation of blast cells overwhelm the bone
marrow and begin filtrating into the peripheral blood

A

Acute Leukemia

  • Cause unknown in most cases, but can be linked to genetics along
    with drug and environmental causes
  • Currently believed to rise from multiple genetic mutations
    influencing the stem cells
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2
Q

General ALL:

Normo- normo- anemia, neutropenia, & thrombocytopenia due to
bone marrow __________.

A

overcrowding

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3
Q

General ALL:

Many ______________ observed in majority of patients, but not all! –
variable morphologic presentation, depending upon subtype.

A

lymphoblasts

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4
Q

General ALL:

increased #s smudge cells! ( > ____ / 100 WBCs)

A

12

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5
Q

General ALL:

  • Splenomegaly and hepatomegaly possible
  • Bone pain from infiltration
  • Lymphoblasts can be observed in ____
A

CSF

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6
Q

ALL accounts for ___% of childhood leukemias.

-peak incidence is between
____ years of age
-rare in adults and have a poor prognosis

A

75

2-5

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7
Q

ALL can be classified as __-ALL or __-ALL

A

B, T

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8
Q

What is the most common lymphoblast size?

A
  • Small size is most common with distinct nucleoli
  • Large type is 2-3 times larger than lymphocyte with distinct nucleoli.
    (May be confused with myeloblasts)
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9
Q

Mature B-ALL Immunophenotype?

A

movement of cytoplasmic CD22 to Surface (sCD 22)

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10
Q

CD10 – _______ (most common)

A

CALLA

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11
Q

Majority of T-ALL have mutations involving the
________ gene, which alters T cell development.

A

NOTCH1

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12
Q

What genetics findings relate to the worst prognosis?

A

B-ALL with the t(9;22); BCR-ABL1 mutation
(Philadelphia chromosome-positive ALL)

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13
Q

Which translocation has an excellent prognosis in children?

A

B-ALL with t(12;21);ETV6-RINX1

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14
Q

________________ is common in B-ALLs with good prognosis in children, bad for adults.

A

Hyperdiploidy

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15
Q

Hypodiploidy is rare with bad prognosis in…

A

both adults and children

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16
Q

What is the prognosis for T-cell ALL?

A

poor

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17
Q

T-cell ALL is more common in….

A

adult and late teen males

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18
Q

T-cell ALL is frequently seen with ___________ mass in teenaged boys.

A

mediastinal mass (thymomegaly)

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19
Q

How is T-cell ALL treated?

A

very aggressive chemo

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20
Q

T-cell ALL will be positive for what cell markers?

A

CD2, (CD3, CD4,)* CD5, & CD8

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21
Q

T-cell All is acid phosphatase ___________.

A

positive

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22
Q

Treatment options for ALLs?

A
  • Chemotherapy administered directly into CSF
  • Methotrexate, vincristine, daunorubicin
  • Monoclonal antibodies (anti-CD20) are assuming a role in lead
    therapy
  • Imatinib, used to treat chronic leukemias
  • BMT or SCT last line of treatment (but can be curative in some pt.
    populations)
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23
Q

Three Chronic Lymphoproliferative
Leukemias

A
    1. Chronic Lymphocytic Leukemia (CLL)
    1. Prolymphocytic Leukemia (PLL)
    1. Hairy Cell Leukemia (HCL)
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24
Q

CLL/SLL onset =

A

insidious, asymptomatic.

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25
CLL/SLL: How is it usually diagnosed?
Typically, diagnosis made during exam for other problems (fatigue, weight loss, lymphadenopathy, &/or splenomegaly)!
26
True or false? CLL/SLL rarely transforms into acute form.
true
27
CLL/SLL: Even with therapy, median survival is ____years.
10
28
CLL/SLL is more common in...
older patients and males
29
What is the most common leukemia in elderly patients?
CLL/SLL
30
CLL/SLL Many ________ cells in peripheral blood due to marked cytoplasmic fragility (also seen in ALLs.)***
smudge
31
CLL/SLL Lab Findings: Report smudge cells as # per ____ WBCs diffed
100
32
CLL/SLL Lab Findings: To ↓ # smudge cells & make diff easier, add...
2 drops 7% B.B. albumin to 0.5 mL whole anticoagulated blood before making smear (always make a note of this!)***
33
CLL/SLL Lab Findings: Bone marrow PAS + for what substance?
glycogen
34
CLL/SLL Lab Findings: 95% cases are B-cell; CD ___________
5, 19(know this, for all B-cell), 20, & 23 +
35
CLL/SLL Lab Findings: 5% cases are T-cell; CD ____________
2, 3, 4, 5, 6, 8 +
36
Most common leukemia/lymphoma in Western Hemisphere.
Chronic Lymphocytic Leukemia (CLL) and Small Lymphocytic Lymphoma (SLL)
37
* Malignant, monoclonal proliferation of small lymphoid cells * Mature-looking, but functionally immature, B cells.
Chronic Lymphocytic Leukemia (CLL) and Small Lymphocytic Lymphoma (SLL)
38
CLL/SLL dont respond to Ag. why?
cells are too immature
39
CLL/SLL positive for CD...
CD5, *CD19, CD20, CD23,* IgM and IgD+ B-cells
40
CLL/SLL diagnosis based on...
Monoclonal B-lymphs > 5,000/uL (normal range for ALC in adults = 1,000-4,000/uL.)
41
SLL involves lymph nodes and ________ infiltration
organ
42
How do lymphs usually appear with CLL/SLL?
mature and mostly normal * Rarely, look immature & abnormal. * Course chromatin and scant cytoplasm * Appear “hypermature” (some have “soccer-ball appearance”!)
43
Prognosis for CLL?
based upon genetic mutations
44
Treatment for CLL?
* Chemotherapy & leukopheresis (to reduce peripheral blood “burden”). * Due to indolent course, use the so-called "watchful waiting” approach. * BMT as last resort.
45
Rare, mature lymphoid leukemia (both B- and T-cell involvement)
Prolymphocytic Leukemia (PLL)
46
Lymph node involvement is mostly seen with __-cell PLL
T
47
Prolymphocytic Leukemia (PLL): Diagnosis requires_____% prolymphocytes
>55
48
PLL: Immature-looking, but functionally intermediate maturity, prolymphocyte ___ cells in peripheral blood.
B
49
PLL: Large ______, moderately coarse chromatin, 1-2 large nucleoli, agranular & lightly basophilic cytoplasm.
pros
50
“punched out” nucleolus is characteristic finding
Prolymphocytic Leukemia (PLL)
51
Prolymphocytic Leukemia (PLL) is most common in...
men in their 60's
52
Prognosis for PLL?
Very poor prognosis, mean survival ~ 3 years w/ B-cell
53
* Absolute lymphocytosis in peripheral blood * ALC = 25,000 - 1,000,000/uL! * Cells will look more mature than lymphoblasts, but yet more immature than a regular “resting lymph”.
Prolymphocytic Leukemia (PLL)
54
Prolymphocytic Leukemia (PLL) is CD...
*CD20, CD19, CD22*, and FMC7
55
Clinical symptoms with PLL?
* Acute onset of common symptoms * Also gross splenomegaly with sweats, & fevers, & weight loss * Presence of sweats & fevers make it important to rule out HIV!
56
Hairy Cell Leukemia (HCL) * Malignant, monoclonal proliferation * Atypical-looking, but functionally intermediate to fully mature, ___-cells in with “hairy” cytoplasmic projections
B
57
"Harry cells" are usually found in...
bone marrow or spleen, sometimes found in peripheral blood, rare in lymph nodes.
58
Large lymphs with big nuclei (round, oval, or indented), pale blue cytoplasm with unique, hair-like surface projections.
Hairy Cell Leukemia (HCL)
59
Hairy Cell Leukemia (HCL) is more common in...
middle-aged men Overall Rare; accounts for only 2% of all leukemias.
60
Hairy Cell Leukemia (HCL): * Characteristic pancytopenia & “__________” on bone marrow aspiration.
dry tap
61
What cells are TRAP positive?***
Harry cells. * Cells contain unusual isoenzyme of acid phosphatase that is not inhibited by tartaric acid: Tartrate-Resistant Acid Phosphatase. -Other lymphoid cells are TRAP neg.
62
Hairy Cell Leukemia (HCL) is PAS, SBB, and MPO _________.
negative
63
HCL is positive for CD...
CD19, CD20, CD22, CD25, and CD103
64
Clinical symptoms of HCL?
* Malaise & fatigue * Hugely enlarged spleen (in 90% patients).
65
Treatment for HCL?
* Considered completely incurable in the past * Now, however, it’s relatively easy to control with chemo. & splenectomy * Good remission rates possible.