CML ICD

Question 1

What are the four types of myeloid cells?

Answer 1

Neutrophils, Monocytes, Eosinophils, and Basophils

Question 2

What are the three types of lymphoid cells?

Answer 2

B, T, and NK Cells

Question 3

The division of multipotential hematopoetic stem cells into myeloid and lymphoid progenitors

Answer 3

Hematopoiesis

Question 4

Myeloid progenitor cells differentiate into what 6 things?

Answer 4

Monocytes, neutrophils, eosinophils, basophils, platelets, and erythrocytes

Question 5

Lymphoid progenitor cells differentiate into

Answer 5

T lymphocytes and B lymphocytes

Question 6

A myeloproliferative disorder characterized by increased proliferation of the granulocytic lineage cells without the loss of their capacity to differentiate

Answer 6

Chronic Myelogenous Leukemia (CML)

Question 7

What percentage of all leukemias that occur in adults are CML?

Answer 7

20%

Question 8

What is the incidence of CML? Is it more common in men or women?

Answer 8

1-1.5/100,000

More common in men

Question 9

CML most commonly affects adults in

Answer 9

Middle Age

Question 10

Characterized by fatigue (34%), weightloss (20%), and early satiety

-often discovered incidentally by complete blood cell count (CBC)

Answer 10

CML

Question 11

What physical characteristic was noted (historically) on >90% of CML patients at diagnosis?

Answer 11

Splenomegaly

Question 12

Abnormal enlargement of the spleen

-The most common physical finding in patients with CML

Answer 12

Splenomegaly

Question 13

Spleen size correlates with the

Answer 13

Peripheral blood granulocyte counts

Question 14

Alterations in hematopoeitic stem cells causes them to leave the bone marrow and take up residence in the spleen, leading to

Answer 14

Extramedullary Hematopoiesis

Question 15

Production of blood cells components OUTSIDE of the bone marrow

Answer 15

Extramedullary Hematopoiesis

Question 16

In the 1960’s, Nowell and Hungerford identified aminute chromosome associated with

Answer 16

CML

Question 17

A small version of chromosome 22 that appears to have a MUCH smaller long arm

-characteristic of CML

Answer 17

Philadelphia Chromosome

Question 18

In 1973, Dr. Jane Rowley determined that the Philadelphia Chromosome was generated by a

Answer 18

Balanced (reciprocal) Translocation between chromosomes 9 and 22

Question 19

With balanced translocations, the individual is often

Answer 19

Phenotypically normal

Question 20

If CML is a balanced translocation, then why are these patients NOT phenotypically normal?

Answer 20

The balanced translocation creates a chimeric protein

Question 21

What is the name of the chimeric protein generated in the balanced translocation characteristic of CML?

Answer 21

BCR-ABL protein

Question 22

A tyrosine kinase that promotes growth and survival

Answer 22

c-Abl

Question 23

c-Abl helps to regulate the

Answer 23

DNA damage repair response

Question 24

This proteins activity is very tightly regulated

Answer 24

c-Abl

Question 25

Gains a domain from BCR that facilitates dimerization

Answer 25

c-Abl

Question 26

c-Abl gains a domain from BCR that facilitates

Answer 26

Dimerization

Question 27

Whe compared to c-Abl, Bcr-Abl is

Answer 27

Constitutively active

Question 28

The constitutively active Bcr-Abl, then continuously activates

Answer 28

Ras, STAT, an AKT

Question 29

c-Abl is normally located in the

Answer 29

Nucleus

Question 30

Bcr-Abl however is trapped in the

Answer 30

Cytoplasm

Question 31

Drives growth and formation of myeloid progenitors

Answer 31

Bcr-Abl

Question 32

Characterized by proliferation of excess myeloid progenitor cells and increased WBC count -Less severe portion of CML

Answer 32

Chronic Phase

Question 33

The most severe portion of CML characterized by excess massive myeloid and lymphoid progenitors that will no longer differentiate -leads to death

Answer 33

Blast Crisis

Question 34

Bcr-Abl location in the cytoplasm alters the

Answer 34

DNA-damage response

Question 35

CML was first described by

Answer 35

John Hughes Bennet, MD

Question 36

What are two forms of molecular diagnostics in CML?

Answer 36

1. ) Cytogenic analysis | 2. ) FISH

Question 37

Can confirm the presence of the Bcr-Abl protein

Answer 37

RT-PCR

Question 38

A unique family of non-receptor protein-tyrosine kinases that are highly conserved

Answer 38

c-Able (Ableson gene) proteins

Question 39

In the Bcr-Abl fusion protein, there is a GAIN-OF-FUNCTION for the Abl tyrosine kinase such that is is

Answer 39

Constitutionally active

Question 40

What does Bcr stand for?

Answer 40

Breakpoint cluster region

Question 41

A drug that blocks ATP binding to Bcr-Abl and prevents the activity of the chimeric protein

Answer 41

Imatonib