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BMS > CML ICD > Flashcards

CML ICD Flashcards

1
Q

What are the four types of myeloid cells?

A

Neutrophils, Monocytes, Eosinophils, and Basophils

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2
Q

What are the three types of lymphoid cells?

A

B, T, and NK Cells

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3
Q

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

A

Hematopoiesis

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4
Q

Myeloid progenitor cells differentiate into what 6 things?

A

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

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5
Q

Lymphoid progenitor cells differentiate into

A

T lymphocytes and B lymphocytes

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6
Q

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

A

Chronic Myelogenous Leukemia (CML)

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7
Q

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

A

20%

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8
Q

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

A

1-1.5/100,000

More common in men

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9
Q

CML most commonly affects adults in

A

Middle Age

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10
Q

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

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

A

CML

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11
Q

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

A

Splenomegaly

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12
Q

Abnormal enlargement of the spleen

-The most common physical finding in patients with CML

A

Splenomegaly

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13
Q

Spleen size correlates with the

A

Peripheral blood granulocyte counts

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14
Q

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

A

Extramedullary Hematopoiesis

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15
Q

Production of blood cells components OUTSIDE of the bone marrow

A

Extramedullary Hematopoiesis

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16
Q

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

A

CML

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17
Q

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

-characteristic of CML

A

Philadelphia Chromosome

18
Q

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

A

Balanced (reciprocal) Translocation between chromosomes 9 and 22

19
Q

With balanced translocations, the individual is often

A

Phenotypically normal

20
Q

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

A

The balanced translocation creates a chimeric protein

21
Q

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

A

BCR-ABL protein

22
Q

A tyrosine kinase that promotes growth and survival

A

c-Abl

23
Q

c-Abl helps to regulate the

A

DNA damage repair response

24
Q

This proteins activity is very tightly regulated

A

c-Abl

25
Q

Gains a domain from BCR that facilitates dimerization

A

c-Abl

26
Q

c-Abl gains a domain from BCR that facilitates

A

Dimerization

27
Q

Whe compared to c-Abl, Bcr-Abl is

A

Constitutively active

28
Q

The constitutively active Bcr-Abl, then continuously activates

A

Ras, STAT, an AKT

29
Q

c-Abl is normally located in the

A

Nucleus

30
Q

Bcr-Abl however is trapped in the

A

Cytoplasm

31
Q

Drives growth and formation of myeloid progenitors

A

Bcr-Abl

32
Q

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

-Less severe portion of CML

A

Chronic Phase

33
Q

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

-leads to death

A

Blast Crisis

34
Q

Bcr-Abl location in the cytoplasm alters the

A

DNA-damage response

35
Q

CML was first described by

A

John Hughes Bennet, MD

36
Q

What are two forms of molecular diagnostics in CML?

A
  1. ) Cytogenic analysis

2. ) FISH

37
Q

Can confirm the presence of the Bcr-Abl protein

A

RT-PCR

38
Q

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

A

c-Able (Ableson gene) proteins

39
Q

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

A

Constitutionally active

40
Q

What does Bcr stand for?

A

Breakpoint cluster region

41
Q

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

A

Imatonib

BMS (62 decks)

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