CML ICD Flashcards Preview

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Flashcards in CML ICD Deck (41):
1

What are the four types of myeloid cells?

Neutrophils, Monocytes, Eosinophils, and Basophils

2

What are the three types of lymphoid cells?

B, T, and NK Cells

3

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

Hematopoiesis

4

Myeloid progenitor cells differentiate into what 6 things?

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

5

Lymphoid progenitor cells differentiate into

T lymphocytes and B lymphocytes

6

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

Chronic Myelogenous Leukemia (CML)

7

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

20%

8

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

1-1.5/100,000

More common in men

9

CML most commonly affects adults in

Middle Age

10

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

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

CML

11

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

Splenomegaly

12

Abnormal enlargement of the spleen

-The most common physical finding in patients with CML

Splenomegaly

13

Spleen size correlates with the

Peripheral blood granulocyte counts

14

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

Extramedullary Hematopoiesis

15

Production of blood cells components OUTSIDE of the bone marrow

Extramedullary Hematopoiesis

16

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

CML

17

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

-characteristic of CML

Philadelphia Chromosome

18

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

Balanced (reciprocal) Translocation between chromosomes 9 and 22

19

With balanced translocations, the individual is often

Phenotypically normal

20

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

The balanced translocation creates a chimeric protein

21

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

BCR-ABL protein

22

A tyrosine kinase that promotes growth and survival

c-Abl

23

c-Abl helps to regulate the

DNA damage repair response

24

This proteins activity is very tightly regulated

c-Abl

25

Gains a domain from BCR that facilitates dimerization

c-Abl

26

c-Abl gains a domain from BCR that facilitates

Dimerization

27

Whe compared to c-Abl, Bcr-Abl is

Constitutively active

28

The constitutively active Bcr-Abl, then continuously activates

Ras, STAT, an AKT

29

c-Abl is normally located in the

Nucleus

30

Bcr-Abl however is trapped in the

Cytoplasm

31

Drives growth and formation of myeloid progenitors

Bcr-Abl

32

Characterized by proliferation of excess myeloid progenitor cells and increased WBC count

-Less severe portion of CML

Chronic Phase

33

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

-leads to death

Blast Crisis

34

Bcr-Abl location in the cytoplasm alters the

DNA-damage response

35

CML was first described by

John Hughes Bennet, MD

36

What are two forms of molecular diagnostics in CML?

1.) Cytogenic analysis
2.) FISH

37

Can confirm the presence of the Bcr-Abl protein

RT-PCR

38

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

c-Able (Ableson gene) proteins

39

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

Constitutionally active

40

What does Bcr stand for?

Breakpoint cluster region

41

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

Imatonib

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