CLL Cases Dr. Nikcevich 5/14/14

Question 1

CLL

Answer 1

Most common leukemia in adults

Allogenic stem cell transplant only curative therapy

CLL in an uncontrolled accumulation of mature lymphocytes

Mutated Ig Vh genes

Question 2

CLL Diagnosis

Answer 2

Flow CD5+CD19+CD20+CD23+***

DDx mantle cell lymphoma (nasty and aggressive)
–CD5+CD23-***cyclinD1+

The difference between CLL and SLL (small lymphocytic lymphoma) is the tissue phase of CLL

It is often a diagnosis in an asymptomatic patient

Question 3

Autoimmune complications of CLL

Answer 3

AIHA

Coomb’s positive and retic count elevated (can happen w/o disease progressing or during remission)

Pure red cell aplasia
DAT negative and retic count absent

Viral–Parvovirus Tx is do nothing and should get better on its own

Question 4

Staging

Answer 4

Rai 0: lymphocytosis
Rai 1: lymphadenopathy
Rai 2: splenomegaly
Rai 3: HgB < 11 g/dL
Rai 4: platelets < 100K

Question 5

Poor prognostic factors

Answer 5

• Advanced stage at diagnosis
• Rapid lymphocyte doubling time
• Diffuse marrow infiltration
• Advanced age/male gender
• CLL-PLL (secondary PLL)
• Abnormal karyotype
• Elevated Beta2-microgolbulin, soluble CD23
• CD38+ (marker for absence of Hb heavy chain rearrangement) or ZAP-70+

Question 6

CLL cytogenetic abnormalities

Answer 6

Best–>Worst

13q-
Normal karyotype
Trisomy 12
11q-
17p-

Always get FISH studies CLL***

Question 7

What if the lab is unable to perform Ig gene mutation studies?

Answer 7

CD38 may be a surrogate marker for Ig gene mutations

• detect via flow
• CD38+=unmutated Ig genes=poorer prognosis
• CD38-=mutated Ig genes=better prognosis

Question 8

When to treat CLL?

Answer 8

Constitutional symptoms
Progressive symptoms
Progressive lymphadenopathy
Progressive splenomegaly
Progressive bone marrow failure
Autoimmune complications
Richter's transformation or secondary PLL
Your patient is uncomfortable w/ observation

Question 9

Secondary prolymphocytic leukemia

Answer 9

• Richter’s transformation
• New 17p-
• Poor prognostic sign

Question 10

IMBRUVICA (ibrutinib)

Answer 10

Mantle Cell Lymphoma
CLL
Accelerated FDA approval
Inhibits BTK which prevents ongoing clonal proliferation

No renal impairment guidelines*
Pregnancy class D
No indication in pediatric population
Can see initially increased WBC that resolves in time*