Lymphoid Leukemia Application Flashcards Preview

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Flashcards in Lymphoid Leukemia Application Deck (35):
1

What does this patient have?
What demographic?
Normal/abberant immunophenotype?
Poly/monoclonal?
Symptoms?

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Mononucleosis
Teenager
Normal
Polyclonal
Fatigue, pharyngitis, splenohepatomegaly

2

What does this patient have?
What demographics?
Normal/abberant immunophenotype?
Poly/monoclonal?

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CLL
Elderly
Abnormal
Monoclonal

3

What is absolute lymphocytosis?
What is the difference between reactive or neoplastic? (Duration, Morphology, Absolute Count, Associated Clinical Findings, Flow cytometery)

>4,000/microL of lymphocytes
Reactive - Acute Duration, Heterogenous, 10-12K or less, Flow reveals polyclonal
Neoplastic - Chronic Duration, Homogenous (Monotonous), 20-40K, Flow reveals monoclonal

Clinical findings are actually pretty common, specific things such as excessive fatigue and weight loss push towards neoplastic

4

What other viruses/pathogens cause mono-like syndromes?

CMV, Adeno, Acute HIV, Toxo

5

How do we assess B cells for clonality?

Light chain restriction on cell surface (kappa v lambda) via flow or immunohistochem
IgH variable gene PCR - One (mono) vs multiple (poly) rearrangements
Immunophenotypic aberrancy (e.g. CD5 (T-cell marker) expression on B cells)

6

T/NK assessment for clonality?

TCR gene PCR
Immunophenotypic aberrancy

7

Flow assessment for clonality? What's left vs right?

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Left - Polyclonal
Right - Monoclonal

8

What is this cell indicative of disease?
What kind of cell is this?
What would be the next step to characterize these cells?

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ALL
Immature cell (Lymphoblast)
Flow cytometery (determine clonality and lineage)

9

What is a smudge cell count used for?
Is it specific?

Determine CLL
No, also occurs with mono, but it is classically tested

10

What markers are indivative for B-ALL?

CD10+/CD19+ (B-cell specific)/CD34+ (Blast specific))
CD3- (T-cell)/CD20- (Mature B-cell)

11

Where can B-ALL expand to?

CSF
Lymph nodes
Bone marrow
Splenic sinusoids

12

Is hypocellularity a finding with ALL?

Not in bone marrow (hypercellulairty in bone marrow)
Cytopenias would appear in peripheral blood

13

What are good prognostic genetics for B-ALL?
What are bad prognostic genetics?
Where are the karyotypes taken from?

Good: Hyperdiploidy, t(12;21)

Bad: Hypodiploidy, t(9;22),11q23 abnormalities (MLL)

They are taken from the specific aberrant cells, can be compared to a buccal swab which should find normal karyotypes

14

What is a large mediastinal mass from an adolescent male indicates?
What would be a charactersitic immunophenotypic finding of this tumor?

T-ALL
CD3+ phenotype

15

Ratio of B-ALL vs T-ALL

85% to 15%

16

Definition of acute leukemia
Can it involve extramedullary sites?
What are the two big categories and the morphology/immunophenotype?

>20% blasts in the blood or bone marrow
Yes

Myeloid - CD13,14,15,33,117 w/w/o Auer rods
Lymphoid - CD3,CD19,CD20

17

What is the pre-leukemic state of AML?
What are the classification states of AML?

Myelodysplasia - Pre-leukemia
AML w/recurrent cytogenic abnormalities
AML w/myelodysplasia-associated changes
AML/MDS, therapy-related
AML, NOS

18

Prognostic factors in B-ALL

Age (2-10 is good, otherwise bad)
Cytogenetic
Elevated WBC count is poor (>100K/uL)
CSF involvement is poor
Marrow involvement at day 15 is poor

19

Treatment of ALL (stages)

Induction - Initiate remission
Intensification - Keep up remission
CNS Prophylaxis - Chemo doesn't enter the brain
Maintenance

20

How is CNS prophylaxis done for ALL?
What other part of the body needs special concern for ALL?

Irradiation or IT methotrexate

Testes also is protected from chemo

21

Cure rate for Adult vs Pediatric ALL

Causes of differences?

Adult - 50% 
Pediatric - 80-90%

Differences in disease and resillience of the body to handle the chemo

22

What is so special about bispecific T-cell engaging (BiTE) antibody?

Directs cytotoxic T-cells to CD19 expressing B-cells to kill them
Blinatumonab

23

How is prognosis for Philidelphia ALL?
What drug improves it?

Horribly bad (7%)
Dasatinib raises it up to 40%+

24

What is the characteristic cell description for CLL?
What are the immunophenotype of CLL?

"Soccer ball" or "Ginger-snap" 
CD5+/CD19+/CD20+/CD23+ with monoclonality (single light chain)

25

What are the causes of anemia in CLL?

Primarily due to crowding out in bone marrow
Secondarily due to immune-mediated destruction of precursors

26

Prognostic Indicators in CLL/SLL

Good: Low Rai stage, Mutated Ig Heavy Variable (IGHV) gene, Deletion 13q

Bad: High Rai stage, Unmutated IGHV gene, CD38/ZAP70 expression, Deletion 11q,17p 

27

What is the classification criteria for small B cell leukemia/lymphomas with 

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28

"Natural" prognosis based on stage for CLL/SLL

Stage 0 - 150 months
Stage I - 101 months
Stage II - 71 months
Stages III/IV - 9 Months

29

What are CLL/SLL indications to treat?

What do we treat with?

Lab: Rapid lymphocyte doubling time (< 6 months), Hgb < 10g/dL (Stage III), Platelets < 100k/uL

Clinical: Autoimmune anemia or thrombocytopenia, richter transformation (large cell transformation), enlaarging symptomatic lymph nodes, B symptoms

FCR

30

What are we trying to do with 17p CLL patients?

Target B-cell signaling (specifically Rituximab (PI3 Kinase) with a BTK inhibitor)

31

What diseases have atypical lymphocytes +/- lymphocytosis?

CLL/SLL
Leukemic lymphoma (follicular, mantle, etc)
Hairy cell leukemia
Adult T cell leukemia/lymphoma
Sezary

32

Classic presentation of Hairy cell leukemia:
Demographics, symptoms, exam finding, immunophenotyping

40-60 y/o males

Any symptom associated with pancytopenia

Splenomegaly

Flow diagnosis: Bright CD11c and CD22 expression; TRAP+

33

What cells are affected by Sezary syndrome?
Whats the triad?
What is the classical histology?
Which skin condition is it affiliated with?
Potential prognostic complication?

CD4+ T-cell neoplasm

Triad of erythroderma, generalized, lymphadenopathy, circulating sezary cells

Cells have "cerebriform nuclei"

Related to mycosis fungoides

SS/MF may transform to aggressive T-cell tumor

34

What is adult T-cell leukemia/lymphoma associated with cell wise?
What virus is it paired with?
Place in world associated with?
Histological finding?
Lab finding?
Clinical course?

CD4+ Neoplasm

HTLV-1 Associated (Not specific)

Japan, Caribbean, Africa

Flower cell with lymphocytosis

Hypercalcemia

Aggressive

35

What cells are associated with large granular lymphoytic leukemia?

What is the clinical course?

What does it present with?

What other diseases is it associated with?

Cytotoxic T cells, NK cells

Indolent disorder

Neutropenia, anemia, splenomegaly

Autoimmune disorders (rheumatoid arthritis)