Ch17 Chronic Myeloproliferative Disorders

Question 0

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

Answer 0

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

Question 1

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

Answer 1

Chronic myeloifproliferation disorders (CMPD)

Question 2

What are the 4 clinical entities of CMPD?

Answer 2

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

Question 3

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

Answer 3

CML

Question 4

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

Answer 4

PV

Question 5

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

Answer 5

ET

Question 6

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?

Answer 6

IMF

Question 7

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

Answer 7

Splenomegaly and usually extramedullary hematopoesis

Question 8

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

Answer 8

CML - Philadelphia chromosome

Question 9

How can CML be characterized?

Answer 9

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

Question 10

CML accounts for _____ of cases of leukemia in adults

Answer 10

20%

Question 11

What is the median age of diagnosis for CML?

Answer 11

45-55

Question 12

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

Answer 12

CML

Question 13

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

Answer 13

Ph chromosome carrying the BCR-ABL gene

Question 14

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

Answer 14

CML

Question 15

The BRC-ABL fusion gene leads to what?

Answer 15

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

Question 16

Symptoms of CML:

Answer 16

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

Question 17

CML is characterized by 3 phases:

Answer 17

Chronic
Accelerated
Blast

Question 18

Characteristics of chronic phase in CML:

Answer 18

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

Question 19

Characteristics of the accelerated phase in CML:

Answer 19

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

Question 20

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

Answer 20

Blast phase

Question 21

What is the most important lab finding for CML?

Answer 21

Increased WBC count in peripheral blood

Question 22

LAP is ______ in CML and ________ in leukemoid reaction

Answer 22

Decreased; increased

Question 23

What is LAP (leukocyte alkaline phosphate)

Answer 23

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

Question 24

Leukemoid reaction would be more likely to have:

Answer 24

Toxic vacuole Toxic granules in neutrophils Strong positive LAP

Question 25

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

Answer 25

10-19%; >20%

Question 26

Diagnosis criteria for the blast phase:

Answer 26

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

Question 27

Current treatment for all stages of CML:

Answer 27

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

Question 28

What is the function of Imatinib?

Answer 28

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

Question 29

What is the only proven cure for CML?

Answer 29

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

Question 30

What is the M:E ratio in patients with CML

Answer 30

10:1

Question 31

What is the chromosomal abnormality in CML

Answer 31

T(9:22)

Question 32

What is consistence with leukemoid reaction

Answer 32

High WBC count Associated with bacterial function High LAP

Question 33

Characteristics of CML

Answer 33

Insidious onset Mature leukemia cells Found in adults

Question 34

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

Answer 34

Blast

Question 35

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

Answer 35

Ionizing radiation Chemicals and drugs Viruses

Question 36

Most patients are diagnosed in which phase of CML?

Answer 36

Chronic phase (85%)

Question 37

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

Answer 37

20%