Chronic Lymphocytic Leukemia Flashcards

1
Q

How is LPL level detected and what does it mean?

A

RNA expression.

Its function in CLL is unknown
But high levels correlates to Unmutated IGHV genes

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2
Q

What are the 1st-line treatment options for CLL in those

A

Chemo-immunotherapy with options of:

  • FCR
  • FR
  • PCR (Pentostatin, Cyclophosphamide, Rituximab)
  • Bendamustine +/- Rituximab
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3
Q

What is detected at high levels in a poor-prognostic CLL?

A

Higher levels of:

  • LPL
  • CLLU1
  • ZAP70
  • CD38
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4
Q

What is the COMPLEMENT 1 Trial?

A

Chlorambucil+Ofatumumab > Chlorambucil

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5
Q

Tell me about the Binet staging

A

Stage A

- Hb 10g/dL or more + Platelets 100k or more +

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6
Q

What is CLL8

A

FCR>FC

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7
Q

What do you need to worry about with Alemtuzumab?

A

High risk of CMV re-activation

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8
Q

What does CLL10 study tell us?

A

This study confirms the superiority of FCR over BR in younger patients.

> 65yo, outcome similar or both regimens, but less toxic for BR.

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9
Q

What is a tumor flare reaction?

A

Painful LN Enlargement or N enlargement with evidence of local inflammation occurring with treatment initiation

May also be a/w speed enlargement, low-grade fever and/or rash

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10
Q

What is considered a complex karyotype?

A

3 or more unrelated chromosome abnormalities in more than 1 cell on karyotype.

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11
Q

How is CD38 level detected and what does a high level mean?

A

Detected by flow cytometry
High expression –> enhanced cell-cell interaction and/or enzyme activity
- high levels correlated to unmutated IGHV genes (which is a poor prognosticator)

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12
Q

What combination gives the highest:

1) CR
2) ORR
3) Med PFS

A

1) CR = FCR
40-50%
Supported by CLL8, CLL10

2) ORR = FCR = BR
- 98%
- CLL10

3) med PFS = FCR > BR > FC > Obinutuzumab/Chlorambucil
- 60m vs 45m vs 30m vs 27m
- CLL8, CLL10, CLL11

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13
Q

What are the specific Genomic alterations in CLL

A

Del 17p13

  • > 80% with TP53 mutations
  • ultra-high to high risk
  • Deregulation of TP53 pathway and loss of cell-cycle regulation

Del 11q

  • intermediate to high risk
  • Deregulation of ATM, BIRC3 and/or other candidate genes

Trisomy 12

  • 19-20%
  • intermediate prognosis

Del 13q

  • ~55%
  • low risk
  • Deregulation of miR15a/16-1 and/or other candidate genes

SOMATIC GENETIC MUTATIONS INCLUDE:

  • NOTCH1 activating mutations - intermediate to high risk
  • SF3B1 deleterious mutations - intermediate to high risk
  • BIRC3 deleterious mutations - High risk
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14
Q

Tell me about the Rai staging

A

Stage 0

  • Lymphocytosis +
  • Lymphocytes >15k/MCL +
  • 40% lymphocytes in the BM

Stage I

  • Lymphocytosis + lymphocytes >15k/MCL and >40% Lymphocytes in BM
  • Enlarged LN present

Stage II

  • Lymphocytosis
  • Lymphocytes >15k/MCL
  • 40% lymphocytes in BM
  • Hepatomegaly +/- Splenomegaly or both

Stage III

  • Lympohcytosis
  • Lymphocytes > 15k/MCL
  • lymphocytes > 40% in BM
  • hepatomegaly +/- splenomegaly
  • Hb 15k/MCL
  • lymphocytes > 40% in BM
  • Enlarged LN
  • hepatomegaly+/-Splenomegaly
  • Hb
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15
Q

What is IgVH?

A

Immunoglobulin heavy chain variable gene

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16
Q

What is Idelalisib?

A

PI3K inhibitor

17
Q

What is the International Prognostic index for CLL? (CLL-IPI)

A

5 components, predicts for OS:

1) Age
2) Clinical stage
3) del (17p) and/or TP53 mutation
4) IGHV mutation status
5) Beta-2microglobulin level

18
Q

How is ZAP70 level detected and what does it mean?

A

Detected by Flow cytometry or RNA expression

High level means high expression due to enhanced BcR signaling

19
Q

How does IgVH mutation status cor relate with survival?

A

Mutated survival >24 yrs

Unmutated survival 8-9 yrs

20
Q

What is CLL10

A

FCR>BR