CLL

Question 1

Immunophenotype

Answer 1

CD5 CD19 CD20 CD22 CD23 CD200
Kappa/Lambda restriction
FMC7-
CD10 -

Question 2

prognostic factors

Answer 2

bad:
unmutated IGHV
del13q14
trisomy12,
del6q23,
del17p13

good:
del11q23

Question 3

Blood smear

Answer 3

round lymph.
smudge cells

Question 4

MBL

Answer 4

Clonality but < 5K lymp
no lymphadeno./splenomegaly/cytopenia
1-2% to become CLL

Question 5

Flow cytometry prognostic factors

Answer 5

CD49d- most powerful flow-cytometry predictor of OS (not available in labs)
IGHV
ZAP-70
CD38

Question 6

NGS

Answer 6

TP53
NOTCH
SF3B1

Question 7

CLL-IPI

Answer 7

concordance of 70%

age >65
rai >= 1
beta-2 microglobulin ≥3.5
del17p13 or TP53
unmutated IGHV

0–1 Low
2–3 Intermediate
4–6 High
7–10 Very high

Question 8

Diagnosis

Answer 8

FACS
no need usually for BM or lymph biospy
tests for CLL-IPI

Question 9

iwCLL Criteria for initiation of CLL therapy

Answer 9

1. Progressive marrow failure
2. Symptomatic splenomegaly.
3. Symptomatic lymphadenopathy.
4. Autoimmune complications poorly responsive to corticosteroids.
5. Symptomatic extranodal involvement (eg, skin, kidney, lung, spine).
6. B symptoms

Question 10

Ibrutinib common side effects

Answer 10

bleeding 2-3%
AF 11%
HTN 20-30%
Arthralgias

Question 11

Ventoclax common side effects

Answer 11

TLS

Question 12

Acalabrutinib vs ibrutinib

Answer 12

less side effects with acala
cannot use PPi with acala
acal is bid and ibruti is qd
more FU with ibruti and more info with P53 mut

Question 13

When to add obintuzumab

Answer 13

need for rapid response
glomerulonephritis/extranodal diseas

Question 14

role of MRD

Answer 14

important to measure
but, does not change Tx decision

Question 15

IPS-E

Answer 15

The International Prognostic Score for Early-stage CLL
Predicts time to treament:

Unmutated IGHV
Lymphocytes >15,000/microL,
Palpable lymph nodes

Question 16

Tx protocols

Answer 16

Ibrutinib until progression
I +R/G until progression (AYA)
Acalabrutinib +/- G until progression
ven + G for 12 months

Question 17

CR def

Answer 17

lymph< 4K
no B symptoms
no BM failure
lymphedeno. < 1.5 cm
no hepato-splenomegaly

Question 18

PR def

Answer 18

2 of 3
Lymph decrease of > 50%
lymphedenopathy decrease > 50%
decrease of hepato-splenomegaly > 50%

and

PLT > 100K
or
HB 11> g% or > 50% above baseline

Question 19

Ibrutinib AE

Answer 19

AF
Bleeding

Question 20

Acalabrutinib AE

Answer 20

Diarrhea
Rash

Question 21

IGHV mutated %

Answer 21

50%

Question 22

MBL definition

Answer 22

monoclonal lymphocytosis of less than 5K with no lymphadenpathy, organomegaly ,cytopenias or B symp.

Question 23

MBL prevelence

Answer 23

X100 of CLL

Question 24

high/low count MBL

Answer 24

high > 500
low < 500

Question 25

MBL types

Answer 25

CLL like type- CD20 dim/negative atypical CLL type- CD20 bright non-CLL type- CD5 neg

Question 26

MBL associated disease

Answer 26

Infections X4 Progression to CLL 1-2% per year non-hematologic cancer X2

Question 27

MBL monitoring

Answer 27

Twice a year for 2 years then annually

Question 28

Mutated/Unmutated

Answer 28

30-50%/50-70%

Question 29

Time to first treatment

Answer 29

Unmutated Lymphocytes > 15K Palpable lympadenopathy

Question 30

CLL risk in 1st degree relatives

Answer 30

X6-9

Question 31

FISH

Answer 31

del13q14 - Favorable trisomy12, del6q23, del11q23- Unfavorable del17p13- shortest OS

Question 32

Follow up for patients with no need for treatment based on IPI

Answer 32

High-Very high risk- 3-6 months Low -Intermediate risk - 6-12 months

Question 33

Role of MRD

Answer 33

Heavy chain PCR Very sensitive but not yet a clinical tool. Decisions should not be based on results of MRD

Question 34

Venetoclax ramp up

Answer 34

Starting a week after 3rd 1/w Gazyva followed by 20 mg 50 mg 100 mg 200 mg 400 mg each for 1 week

Question 35

Venteoclax+ Ibrutinib

Answer 35

for high risk patients age> 65, del17, del11 or TP53 mutation 2 year fixed duration PFS and OS exceeded 95%

Question 36

Cumulative Illness Rating Scale (CIRS)

Answer 36

0-4 Evaluates comorbidly in each system separately

Question 37

Venetoclax vs. Ibrutinib

Answer 37

Ventoclax- fixed duration, less AF and bleeding, caution with renal failure, need for more intense follow up. Ibrutnib- more infeormaton with P53

Question 38

Options for R/R naïve to novel Tx

Answer 38

try Clinical trial, else: =if (OR(TP53 mut, del17p13), BTKi, choices) choices = {BTKi, Venetoclax, Obinutuzumab)