Flashcards in Chronic Lymphocytic Leukemia Deck (20):
How is LPL level detected and what does it mean?
Its function in CLL is unknown
But high levels correlates to Unmutated IGHV genes
What are the 1st-line treatment options for CLL in those
Chemo-immunotherapy with options of:
- PCR (Pentostatin, Cyclophosphamide, Rituximab)
- Bendamustine +/- Rituximab
What is detected at high levels in a poor-prognostic CLL?
Higher levels of:
What is the COMPLEMENT 1 Trial?
Chlorambucil+Ofatumumab > Chlorambucil
Tell me about the Binet staging
- Hb 10g/dL or more + Platelets 100k or more +
What is CLL8
What do you need to worry about with Alemtuzumab?
High risk of CMV re-activation
What does CLL10 study tell us?
This study confirms the superiority of FCR over BR in younger patients.
>65yo, outcome similar or both regimens, but less toxic for BR.
What is a tumor flare reaction?
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
What is considered a complex karyotype?
3 or more unrelated chromosome abnormalities in more than 1 cell on karyotype.
How is CD38 level detected and what does a high level mean?
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)
What combination gives the highest:
3) Med PFS
1) CR = FCR
Supported by CLL8, CLL10
2) ORR = FCR = BR
3) med PFS = FCR > BR > FC > Obinutuzumab/Chlorambucil
- 60m vs 45m vs 30m vs 27m
- CLL8, CLL10, CLL11
What are the specific Genomic alterations in CLL
- >80% with TP53 mutations
- ultra-high to high risk
- Deregulation of TP53 pathway and loss of cell-cycle regulation
- intermediate to high risk
- Deregulation of ATM, BIRC3 and/or other candidate genes
- intermediate prognosis
- 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
Tell me about the Rai staging
- Lymphocytosis +
- Lymphocytes >15k/MCL +
- 40% lymphocytes in the BM
- Lymphocytosis + lymphocytes >15k/MCL and >40% Lymphocytes in BM
- Enlarged LN present
- Lymphocytes >15k/MCL
- 40% lymphocytes in BM
- Hepatomegaly +/- Splenomegaly or both
- Lymphocytes > 15k/MCL
- lymphocytes > 40% in BM
- hepatomegaly +/- splenomegaly
- Hb 15k/MCL
- lymphocytes > 40% in BM
- Enlarged LN
What is IgVH?
Immunoglobulin heavy chain variable gene
What is Idelalisib?
What is the International Prognostic index for CLL? (CLL-IPI)
5 components, predicts for OS:
2) Clinical stage
3) del (17p) and/or TP53 mutation
4) IGHV mutation status
5) Beta-2microglobulin level
How is ZAP70 level detected and what does it mean?
Detected by Flow cytometry or RNA expression
High level means high expression due to enhanced BcR signaling
How does IgVH mutation status cor relate with survival?
Mutated survival >24 yrs
Unmutated survival 8-9 yrs