Unit1 Chronic Leukemias

Front 1

Excess production of mature cells in the myeloid line is what leukemia?

Back 1

CML

Front 2

Neutrophils, platelets, bands, metamyelocytes, eosinophils, and basophils will all be elevated in what leukemia?

Back 2

CML

Front 3

Why don't CML patients have elevated RBCs?

Back 3

Because RBCs mature more slowly than WBCs

Front 4

What chromosome is associated with CML?

Back 4

Philadelphia chromosome
t(9;22)

Front 5

In leukemia, chromosome 9 has what gene?

Back 5

abl (Abelson leukemia virus)

Front 6

In leukemia, chromosome 22 has what gene?

Back 6

bcr (breakpoint cluster gene)

Front 7

What is the most sensitive test to diagnose the Philadelphia chromosome?

Back 7

PCR

Front 8

The abl protein is what kind of enzyme?

Back 8

Tyrosine kinase - involved in signal transduction

Front 9

The Philadelphia chromosome causes what to happen to tyrosine kinase?

Back 9

It is always phosphorylated (always on)

Front 10

What does the constant phosphorylation of tyrosine kinase cause?

Back 10

Results in cell growth that exceeds apoptosis

Front 11

What bcr-abl protein product is seen in ALL?

Back 11

p190 (190 kDa protein)

Front 12

What bcr-abl protein product is seen in CML?

Back 12

p210 (210 kDa protein)

Front 13

What chromosome is always present in CML?

Back 13

Philadelphia chromosome

Front 14

How many blasts are present in the bone marrow in the chronic phase of CML?

Back 14

Less than 5%

Front 15

What is the range percentage of blasts in the marrow present in accelerated CML?

Back 15

5-20%

Front 16

How many blasts in the bone marrow are present in the blast crisis of CML?

Back 16

More than 20%

Front 17

The blast crisis of CML can be myeloid or lymphoid, true or false?

Back 17

True

Front 18

What is the "old" treatment for CML?

Back 18

High dose interferon
Allogenic stem cell transplant

Front 19

What is the most commonly used drug for CML now?

Back 19

Gleevec (imatinib mesylate)

Front 20

What does Gleevec inhibit?

Back 20

Tyrosine kinase
Signal transduction
Small molecule

Front 21

What are the side effects of Gleevec?

Back 21

Mild nausea & vomiting
Periorbital edema
Pleural effusions

Front 22

What is the MOA of Gleevec?

Back 22

Blocks ATP from binding to protein
Inhibits tyrosine kinase, signal transduction

Front 23

What does Gleevec bind to on GISTs?

Back 23

c-kit

Front 24

What other illnesses can Gleevec treat?

Back 24

GI stromal tumors (GIST)
Hypereosinophilic syndrome

Front 25

What does Gleevec bind to in hypereosinophilic syndrome?

Back 25

PDGFR-alpha

Front 26

What mutation is resistant to Gleevec?

Back 26

T315I

Front 27

What are the second generation tyrosine kinase inhibitors?

Back 27

Dasatinib
Nilotinib

Front 28

What is a myeloid blast crisis of CML treated with?

Back 28

Cytarabine 7 days
Daunorubicin 3 days

Front 29

A lymphoid blast crisis of CML is treated like what other leukemia?

Back 29

ALL

Front 30

CLL is a continuum with what lymphoma?

Back 30

SLL - small lymphocytic lymphoma

Front 31

What does the CBC look like in CLL?

Back 31

Elevated WBCs
Lymphocytic diff
Normal Hgb, platelets

Front 32

Bone marrow is not needed for the diagnosis of what leukemia?

Back 32

CLL

Front 33

What test is used to diagnose CLL?

Back 33

Flow cytometry

Front 34

What CDs are on normal B cells?

Back 34

19, 23

Front 35

What CDs are on normal T cells?

Back 35

5

Front 36

If CD5 is found on a B cell, what does this indicate?

Back 36

CLL

Front 37

What light chains are restricted in CLL?

Back 37

Kappa or lambda, not both

Front 38

The life expectancy for CLL with only lymphocytosis is what w/o treatment?

Back 38

10 or more years

Front 39

The prognosis for CLL with lymphocytosis and lymphadenopathy is what w/o treatment?

Back 39

8 or more years

Front 40

The prognosis for CLL with lymphocytosis and splenomegaly is what w/o treatment?

Back 40

about 5 years

Front 41

The prognosis for CLL with Hgb less than 11 OR platelets less than 100K is what w/o treatment?

Back 41

8-12 months

Front 42

What are the indications for treatment in CLL?

Back 42

Symptomatic lymphadenopathy
Symptomatic splenomegaly
Anemia, thrombocytopenia

Front 43

Absolute white cell count is an indication to treat CLL, true or false?

Back 43

False

Front 44

What hemolytic illnesses are frequently seen in CLL patients?

Back 44

Autoimmune hemolytic anemia
Immune thrombocytopenic purpura

Front 45

How do you tell if a CLL patient's thrombocytopenia is from ITP or stage IV disease?

Back 45

Bone marrow

Front 46

Development of diffuse large B cell lymphoma from one CLL clone is known as what?

Back 46

Richter's transformation

Front 47

PET scan is necessary to distinguish between what?

Back 47

CLL and DLBCL
DLBCL is PET avid

Front 48

What is DLBCL treated with?

Back 48

R-CHOP

Front 49

Transformation of CLL to prolymphocytic leukemia is shown by what on the blood smear?

Back 49

over 55% prolymphocytes

Front 50

What drugs are used in the treatment of CLL?

Back 50

Purine analog chemo
Fludarabine
Pentostatin

Front 51

What is a significant risk of the first cycle of CLL treatment?

Back 51

Tumor lysis syndrome

Front 52

What aberrant T-cell expression is found in hairy cell leukemia?

Back 52

CD103

Front 53

Hairy cell leukemia is what stain positive?

Back 53

TRAP

Front 54

What is hairy cell clinically notable for?

Back 54

very large spleens
dry taps on bone marrow

Front 55

Hairy cell leukemia comprises what percentage of all leukemias?

Back 55

2%

Front 56

What does the marrow look like in hairy cell leukemia?

Back 56

Lots of fibrosis

Front 57

What is the treatment for hairy cell leukemia?

Back 57

Purine analogs
Cladribine