Nikcevich- CLL Cases

Question 1

What is the most common leukemia in adults? What is the median age of diagnosis and survival?

Answer 1

Chronic lymphocytic leukemia

65

9 yrs

Question 2

What is the only curative therapy for CLL?

Answer 2

allogenic stem cell transplant

Question 3

What is CLL?

Answer 3

Uncontrolled clonal accumulation of mature lymphocytes

assoicated w/ mutated IgVh genes

Question 4

If you were using flow cytometry to diagnose a case of CLL what CD markers would you be looking for?

Answer 4

CD5
CD19
CD20
CD23

Question 5

What CDs are associated w/ mantle cell lymphoma?

Answer 5

CD5
CD23
cyclin D

Question 6

What is a DDX for CLL?

Answer 6

Follicular lymphoma
mantle cell lymphoma (distinguished by CD)
Prolymphocytic leukemia
SLVL
HCL

Question 7

CLL is often an _________dx in a _______pt.

Answer 7

incidental

asymptomatic pt

Question 8

Progressive adenopathy and bone marrow involvement correlates with what sxs in CLL?

Answer 8

fatigue, malaie, weight loss, fevers

Severe cytopenias, increased risk of infection

Question 9

What are the long term complicatoins of CLL?

Answer 9

Autoimmne sequelae

Richter’s transformation

Question 10

What autoimmune complications are associated w/ CLL?

Answer 10

AIHA
Pure red cell aplasia
ITP
Neutropenia

Question 11

What are the Rai system stages?

Answer 11

0-lymphocytosis
1-lymphadenopathy
2- splenomegaly
3- HgB < 11
4- platelets < 100K

Question 12

What are the Binet system stages?

Answer 12

A- lymphocytosis and 1-3 sites lymphadenopathy
B- lymphocytosis w/ >3 sites lymphadenopathy
C- lymhphocytois + anemia/thrombocytosis

Question 13

What is the median survival for CLL?

Answer 13

> 10 years

Question 14

What are poor prognostic factors for CLL?

Answer 14

Advanced stage at dx
Rapid lymphocyte doubling time
diffuse marrow infiltration
advanced age/M
Elevated b2 microglobulin, soluble CD23

Question 15

What CD is associated w/ poor prognosis?

Answer 15

CD23

ZAP 70

Question 16

What are cytogenetic abnormalities are associated w/ CLL?

Answer 16

13q
Trisoy 12
11q
17p

**ALWAYS get FISH studies w/ CLL

Question 17

CD38 can be a surrogate marker for Ig gene mutations. You can detect via flow cytometry!
CD38+ and CD38- are associated w/ what prognosis? survival?

Answer 17

CD38+ unmutated Ig gene mutations= poor prognosis (9 year survival)

CD38- mutated Ig genes= better prognosis (>20 yrs survival)

Question 18

ZAP 70 expression correlates w/ what CD expression/prognosis?

Answer 18

CD38 expression
POOR prognosis

**correlates w/ IgVh gene mutations

Question 19

When should you treat CLL?

Answer 19

When there is progressive lymphocytosis, lymphadenopathy, splenomegaly, obne marrow failure

Question 20

What are the tx options for CLL?

Answer 20

Corticosteroids

Alkylating agents

Question 21

What nucleoside analogs can be used to tx CLL?

Answer 21

FLudarabine

Pentostatin

Question 22

What monoclonal antibodies can be used to tx CLL?

Answer 22

Rituximab (anti-CD20)

Ofatumumuab (anti-CD 20)

Question 23

What combination chemo is used to tx CLL?

Answer 23

PCR

FCR

Question 24

How do you treat younger pts w/ CLL?

Answer 24

Clinical trial
PCR/FCR
Bendamustine plus rituximab
Ibrutinib

Question 25

How do you treat older, more frail pts?

Answer 25

Chlorambucil +/- prednisone
Bendamustine plus rituximab
Ibrutinib

Question 26

What is the only curative modality for CLL?

Answer 26

allogenic transplant
need matched-sib donor
substantial morbidity/mortality

Question 27

What is the price of curing CLL?

Answer 27

GVHD