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Hematology > Ch17 Chronic Myeloproliferative Disorders > Flashcards

Ch17 Chronic Myeloproliferative Disorders Flashcards

0
Q

Clonal expansion of a hematopoietic pluripotent stem cell results in what?

A

BM hyperplasia related to a dominant expansion of 1 or more myeloid cell lines

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1
Q

What disorder is a heterogenous group of diseases originates from the clinal expansion of a hematopoietic pluripotent stem cell?

A

Chronic myeloifproliferation disorders (CMPD)

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2
Q

What are the 4 clinical entities of CMPD?

A

Chronic myelogenous leukemia (CML)
Polycythemia Vera (PV)
Essential thrombocythemia (ET)
Chronic idiopathic myelofibrosis (IMF)

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3
Q

Which entity of CMPD has the BM show a granulocytes hyperplasia with a dominant increase in granulocytes in the peripheral blood?

A

CML

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4
Q

Which entity of CMPD is am erythroid hyperplasia in the BM accompanied by an increase in erythrocytes in the peripheral blood?

A

PV

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5
Q

Which entity of CMPD is a megakaryocyte that dominantly increased in th marrow and platelets In the peripheral blood

A

ET

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6
Q

Which entity of CMPD would seem to be the exception because it is characterized by a decrease in most cells in the peripheral blood, especially in the late stages?

A

IMF

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7
Q

What is common to all (99%) of CMPD?

A

Splenomegaly and usually extramedullary hematopoesis

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8
Q

What entity of CMPD is associated with a consistent chromosomal abnormality?

A

CML - Philadelphia chromosome

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9
Q

How can CML be characterized?

A

A marked increase of granulocytes in the peripheral blood, including neutrophils with immature forms, eosinophils, and basophils, as well as marked granulocytic hyperplasia of the BM

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10
Q

CML accounts for _____ of cases of leukemia in adults

A

20%

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11
Q

What is the median age of diagnosis for CML?

A

45-55

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12
Q

A clonal stem disorder characterized by the presence of the Ph chromosome:

A

CML

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13
Q

It is the presence of _________ that gives the abnormal CML clone it’s growth advantage over normal cells and allows them to replace BM elements

A

Ph chromosome carrying the BCR-ABL gene

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14
Q

In ____ immature granulocytes fill the BM and are released prematurely into the peripheral blood in various stages of maturations

A

CML

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15
Q

The BRC-ABL fusion gene leads to what?

A

Defective adhesion of the CML blonde to BM, resulting in early release of immature forms into the blood stream

16
Q

Symptoms of CML:

A 
General malaise
Fullness of upper abdomen
Loss of appetite 
Bone tenderness
Night sweats and weight loss 
Symptoms associated with anemia
17
Q

CML is characterized by 3 phases:

A

Chronic
Accelerated
Blast

18
Q

Characteristics of chronic phase in CML:

A

85% of patients
Stable for several years
Responsive to conventional chemotherapy

19
Q

Characteristics of the accelerated phase in CML:

A 
Unexplained fevers
Severe weight loss
Progressive leukocyte sis
Bleeding
Thrombosis
Infections 
Bone and joint pain
20
Q

What phase of CML is represented by the conversion of CML to an aggressive form of acute leukemia?

A

Blast phase

21
Q

What is the most important lab finding for CML?

A

Increased WBC count in peripheral blood

22
Q

LAP is ______ in CML and ________ in leukemoid reaction

A

Decreased; increased

23
Q

What is LAP (leukocyte alkaline phosphate)

A

An enzyme present in normal neutrophils, but absent in malignant neutrophils

24
Q

Leukemoid reaction would be more likely to have:

A

Toxic vacuole
Toxic granules in neutrophils
Strong positive LAP

25
Q

In the accelerated phase on CML blasts are ____ and basophils are _____ in the peripheral blood

A

10-19%; >20%

26
Q

Diagnosis criteria for the blast phase:

A

Blasts >20%
Extra medullary blast proliferation
Cluster of blasts in BM biopsy

27
Q

Current treatment for all stages of CML:

A

Imatinib mesylate (Gleevec)-drug directed against a specifics molecular target

28
Q

What is the function of Imatinib?

A

To directly inhibit the mutant tyrosine kinase activity of the BCR-ABL function gene

29
Q

What is the only proven cure for CML?

A

Allogenic BM transplant with an HLA-matched related or unrelated donor

30
Q

What is the M:E ratio in patients with CML

A

10:1

31
Q

What is the chromosomal abnormality in CML

A

T(9:22)

32
Q

What is consistence with leukemoid reaction

A

High WBC count
Associated with bacterial function
High LAP

33
Q

Characteristics of CML

A

Insidious onset
Mature leukemia cells
Found in adults

34
Q

What phase of CML carries the worst prognosis and is generally unresponsive to treatment?

A

Blast

35
Q

Environmental factors that are associated with an increased risk of developing leukemia include:

A

Ionizing radiation
Chemicals and drugs
Viruses

36
Q

Most patients are diagnosed in which phase of CML?

A

Chronic phase (85%)

37
Q

The blast phase of CML is defined by ____ percent of blasts found in the peripheral blood or BM

A

20%

Hematology (10 decks)

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