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Heme/Lymph > Lymphoid Leukemia Application > Flashcards

Lymphoid Leukemia Application Flashcards

1
Q

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

A

Mononucleosis
Teenager
Normal
Polyclonal
Fatigue, pharyngitis, splenohepatomegaly

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2
Q

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

A

CLL
Elderly
Abnormal
Monoclonal

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3
Q

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

A

>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

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4
Q

What other viruses/pathogens cause mono-like syndromes?

A

CMV, Adeno, Acute HIV, Toxo

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5
Q

How do we assess B cells for clonality?

A 
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)
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6
Q

T/NK assessment for clonality?

A

TCR gene PCR
Immunophenotypic aberrancy

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7
Q

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

A

Left - Polyclonal
Right - Monoclonal

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8
Q

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

A 
ALL
Immature cell (Lymphoblast)
Flow cytometery (determine clonality and lineage)
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9
Q

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

A

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

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10
Q

What markers are indivative for B-ALL?

A

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

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11
Q

Where can B-ALL expand to?

A

CSF
Lymph nodes
Bone marrow
Splenic sinusoids

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12
Q

Is hypocellularity a finding with ALL?

A

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

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13
Q

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

A

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

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14
Q

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

A

T-ALL
CD3+ phenotype

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15
Q

Ratio of B-ALL vs T-ALL

A

85% to 15%

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16
Q

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

A

>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
Q

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

A

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

18
Q

Prognostic factors in B-ALL

A

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
Q

Treatment of ALL (stages)

A

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

20
Q

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

A

Irradiation or IT methotrexate

Testes also is protected from chemo

21
Q

Cure rate for Adult vs Pediatric ALL

Causes of differences?

A

Adult - 50%
Pediatric - 80-90%

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

22
Q

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

A

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

23
Q

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

A 
Horribly bad (7%)
Dasatinib raises it up to 40%+
24
Q

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

A

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

25
Q

What are the causes of anemia in CLL?

A

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

26
Q

Prognostic Indicators in CLL/SLL

A

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
Q

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

A
28
Q

“Natural” prognosis based on stage for CLL/SLL

A

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

29
Q

What are CLL/SLL indications to treat?

What do we treat with?

A

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
Q

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

A

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

31
Q

What diseases have atypical lymphocytes +/- lymphocytosis?

A

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

32
Q

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

A

40-60 y/o males

Any symptom associated with pancytopenia

Splenomegaly

Flow diagnosis: Bright CD11c and CD22 expression; TRAP+

33
Q

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?

A

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
Q

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?

A

CD4+ Neoplasm

HTLV-1 Associated (Not specific)

Japan, Caribbean, Africa

Flower cell with lymphocytosis

Hypercalcemia

Aggressive

35
Q

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?

A

Cytotoxic T cells, NK cells

Indolent disorder

Neutropenia, anemia, splenomegaly

Autoimmune disorders (rheumatoid arthritis)

Heme/Lymph (18 decks)

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