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Pathology - Blood Module > Chronic Myelogenous Leukemia > Flashcards

Chronic Myelogenous Leukemia Flashcards

1
Q

What happens in myeloproliferative disorders?

A

Increased production of one or more totes of blood cells

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2
Q

Myeloproliferative disorders can transform to _______________ (acute myeloid leukaemia/chronic lymphocytic leukaemia)

A

Acute myeloid leukaemia

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3
Q

Chronic myelogenous leukaemia (a type of MPD) can transform into _______________

A

Acute lymphocytic leukaemia

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4
Q

The common pathogenic feature of MPDs is the presence of mutated, activated ___________ that lead to growth factor independence.

A

Tyrosine kinases

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5
Q

MPDs do not impair differentiation so the most common effect is an __________ (increase/decrease) in the production of one or more mature blood elements

A

Increase

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6
Q

What are the main features of MPDs?

A
  1. Increased proliferation in bone marrow
  2. Homing of neoplastic stem cells to secondary hematopoietic organs —> Extramedullary hematopoiesis
  3. Transformation to a spent phase (marrow fibrosis and peripheral blood cytopenias)
  4. Variable transformation to acute leukaemia
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7
Q

Systemic mastocytosis is a MPD that is associated with mutations in the _____ tyrosine kinase

A

KIT

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8
Q

Chronic myelogenous leukaemia is characterized by presence of chimeric __________ gene

A

BCR-ABL gene

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9
Q

BCL gene is on chromosome no. _____

A

22

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10
Q

ABL gene is on chromosome no. ____

A

9

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11
Q

In more than 90% of the cases, BCR-ABL is created by a _______________

A

Reciprocal translocation (9;22) (q34;q11)

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12
Q

Reciprocal translocation (9;22) (q34;q11) is also called the _______________ chromosome.

A

Philadelphia

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13
Q

The cell of origin is a ____________(pluripotent/multipotent) hematopoietic stem cell in CML.

A

Pluripotent

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14
Q

The _____ moiety of BCR-ABL contains a dimerization domain that self associates, leading to the activation of the other tyrosine kinase moiety in CML.

A

ABL

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15
Q

The marrow is markedly __________ (hypocellular/hypercellular) in CML.

A

Hypercellular

Because of massively increased numbers of maturing granulocytic precursors

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16
Q

___________ (increased/decreased) proportion of eosinophils and basophils is found in CML.

A

Increased

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17
Q

Megakaryocytes are ___________ (increased/decreased) and usually include
small, dysplastic forms and so are platelets in CML

A

Increased

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18
Q

Erythroid progenitors are present in

normal or mildly __________ (increased/decreased) numbers in CML.

A

decreased

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19
Q

A characteristic finding is the presence of scattered macrophages with abundant wrinkled, green-blue cytoplasm so-called _______________ in CML.

A

Sea green histiocytes

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20
Q

_________ (Increased/decreased) deposition of reticulin is typical in CML

A

Increased

21
Q

Overt marrow fibrosis occurs ______ (early/late) in the course of CML.

A

late

22
Q

The blood reveals a leukocytosis, often exceeding __________ (50,000/100,000) cells/mm3 which consists predominantly of neutrophils, band forms, metamyelocytes, myelocytes, eosinophils, and basophils in CML.

A

100,000 cells/mm3

23
Q

Blasts usually make up less than ___% of the circulating cells in CML

A

10

24
Q

The spleen is often greatly enlarged as a result of extensive __________ (hemolysis/extramedullary hematopoiesis) and often contains infarcts of varying age in CML

A

extramedullary hematopoiesis

25
Q

_______________ can also produce mild hepatomegaly and lymphadenopathy

A

Extramedullary hematopoiesis

26
Q

CML is primarily a disease of _________ (adults/children)

A

adults

27
Q

The peak incidence is in the ______ (5th-6th/3th-4th) decades of life in CML

A

5th-6th

28
Q

What are the symptoms of CML?

A 
Mild-to-moderate anemia
Hypermetabolism
Fatigability
Weakness
Weight loss
Anorexia.
Dragging sensation in the abdomen
Acute onset of left upper quadrant pain
29
Q

Why is hypermetabolism going on in patient with CML

A

Due to increased cell turnover leading to fatigabililty

30
Q

What can be the reason of dragging sensation of abdomen in CML?

A

enlarged spleen

31
Q

What is the reason of acute onset of left upper quadrant pain in CML?

A

Splenic infarcts

32
Q

The natural history is one of slow progression; even

without treatment, the median survival is about _________ (1 year/3 years)

A

3 years

33
Q

After a variable period averaging 3 years, about 50% of

patients enter an ________ (accelerated/spent) phase of CML

A

accelerated

34
Q

What happens in accelerated phase of CML?

A

Increasing anemia and thrombocytopenia sometimes accompanied with a rise in basophils.

35
Q

What is the duration of accelerated phase of CML?

A

After 6-12 months, the accelerated phase terminates in acute leukemia (blast crisis)

36
Q

Give examples of additional clonal cytogenetic abnormalities that often appear in accelerated phase of CML.

A

Trisomy 8
Isochromosome 17q
Duplication of the Ph chromosome

37
Q

In the other ___% of patients, blast crises occur

abruptly without an accelerated phase in CML

A

50%

38
Q

In 70% of crises, the blasts are of _________ (myeloid/lymphoid) origin in CML.

A

myeloid

39
Q

The remaining 30% blasts are of _________ (pre–B cell/pre–T cell) origin in lymphoid blast crises of CML

A

pre–B cell origin

40
Q

What is the evidence that CML originates from pluripotent stem cell?

A

Both type of blast crises occurs (myeloid and lymphoid)

41
Q

Acute leukemia stems from two complementary mutations. What are these mutations?

A

Mutation in transcriptional factor and tyrosine kinase

42
Q

Blast crises occurs due to the mutation of ____________ in CML

A

transcription factor

43
Q

In lymphoid blast crisis, 85% of cases are associated with mutations that interfere with the activity of
________.

A

Ikaros

44
Q

What is Ikaros?

A

A transcription factor that regulates the differentiation of hematopoietic progenitors

45
Q

Ikaros mutations are also seen in BCR-ABL positive _________

A

B-ALL

46
Q

Treatment with _________ inhibitors results in sustained hematologic remissions in greater than 90% of patients.

A

BCR-ABL inhibitors

47
Q

For relatively young patients, ______________ performed in the stable phase is curative in about 75% of cases.

A

Hematopoietic stem cell transplantation

48
Q

The treatment is less effective in __________ (stable/accelerated) phase

A

accelerated

49
Q

What exactly is the function of BCR-ABL inhibitors?

A
  1. They decrease the number of BCR-ABL-positive cells in the marrow
  2. They decrease the rate at which these cells acquire
    mutations
  3. They control blood counts and decrease the risk of transformation to the accelerated phase and blast crisis.

Pathology - Blood Module (20 decks)

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