Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Path U2 > CH6 - 4) Myeloproliferative Disorders > Flashcards

CH6 - 4) Myeloproliferative Disorders Flashcards

1
Q

What are myeloproliferative disorders?

A

Neoplastic proliferation of mature cells of myeloid lineage; disease of late adulthood (average age is 50-60 years)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What does myeloproliferative disorders result in?

A

high WBC count with hypercellular bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How are myeloproliferative disorders characterized?

A

Cells of all myeloid lineages are increased; classified based on the dominant myeloid cell produced

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the complications for myeloproliferative disorders?

A

1) Increased risk for hyperuricemia and gout due to high turnover of cells 2) Progression to marrow fibrosis or transformation to acute leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is chronic myeloid leukemia?

A

Neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors;

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

In chronic myeloid leukemia what are characteristically increased?

A

basophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is chronic myeloid leukemia driven by?

A

driven by t(9:22) - Philadelphia chromosome - which generates a BCR-ABL fusion protein with increased tyrosine kinase activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the first line treatment for chronic myeloid leukemia?

A

imatinib,

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What does imatinib do?

A

It blocks tyrosine kinase activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How is splenomegaly related to chronic myeloid leukemia?

A

Splenomegaly is common, enlarging spleen suggests accelerated phase of disease;

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

In chronic myeloid leukemia what usually follows after splenomegaly?

A

transformation to acute leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What can chronic myeloid leukemia transform to?

A

AML in 2/3 of cases or ALL in 1/3 of cases since mutation is in a pluripotent stem cell,

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is a leukemoid reaction?

A

Reactive neutrophilic leukocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How is CML distinguished from a leukemoid reaction (reactive neutrophilic leukocytosis)?

A

Negative LAP stain, inc basophils, t(9;22)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is a LAP stain?

A

leukocyte alkaline phosphatase (LAP) stain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How is a LAP stain related to a leukemoid reaction?

A

granulocytes in a leukemoid reaction are LAP positive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Why are basophils useful in determining the difference between a leukemoid reaction and CML?

A

Basophils are increased in CML and are absent with a leukemoid reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How is t(9;22) useful in determining the difference between a leukemoid reaction and CML?

A

It is present in CML and absent in leukemoid reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is polycythemia vera?

A

Neoplastic proliferation of mature myeloid cells, especially RBCs

20
Q

In polycythemia vera, in addition to the RBC increase what are also increased?

A

Granulocytes and platelets are also increased

21
Q

What mutation is associated with polycythemia vera?

A

JAK2 kinase mutation

22
Q

What are the clinical symptoms for polycythemia vera due to?

A

they are mostly due to hyperviscosity of blood

23
Q

What are the clinical symptoms for polycythemia vera?

A
  1. Blurry vision and headache 2. Increased risk of venous thrombosis (e.g., hepatic vein, portal vein, and dural sinus) 3. Flushed face due to congestion (plethora) 4. Itching, especially after bathing (due to histamine release from increased mast cells)
24
Q

What is plethora in relation to PV?

A

One of the clinical symptoms; flushed face due to congestion

25
Q

Why is there itching in PV?

A

Itching, especially after bathing due to histamine release from increased mast cells

26
Q

What is the treatment for PV?

A

phlebotomy;

27
Q

What is the second-line therapy for PV?

A

hydroxyurea.

28
Q

What happens to a patient with PV who goes intreated?

A

Without treatment, death usually occurs within one year.

29
Q

How can PV be distinguished from reactive polycythemia?

A
  1. In PV, erythropoietin (EPO) levels are decreased, and Sao, is normal. 2. In reactive polycythemia due to high altitude or lung disease, SaO2 is low, and EPO is increased. 3. In reactive polycythemia due to ectopic EPO production from renal cell carcinoma, EPO is high, and Sao2 is normal.
30
Q

What are the EPO and SaO2 levels in PV?

A

EPO is decreased and SaO2 is normal

31
Q

What are the EPO and SaO2 levels in reactive polycythemia due to high altitude or lung disease?

A

EPO is increased and SaO2 is low

32
Q

What are the EPO and SaO2 levels in reactive polycythemia due to renal cell carcinoma?

A

EPO is high, SaO2 is normal

33
Q

What is essential thrombocythemia?

A

Neoplastic proliferation of mature myeloid cells, especially platelets

34
Q

In ET, in addition to the platelets what else is increased?

A

RBCs and granulocytes are also increased.

35
Q

What is mutation is ET associated with?

A

JAK2 kinase mutation

36
Q

What do the symptoms of ET result from?

A

they are related to an increased risk of bleeding and/or thrombosis.

37
Q

What do the symptoms of ET rarely progresses to?

A

marrow fibrosis or acute leukemia

38
Q

In ET what is there no significant risk for?

A

hyperuricemia or gout

39
Q

What is myelofibrosis?

A

It is neoplastic proliferation of mature myeloid cells, especially megakaryocytes

40
Q

What mutation is associated with myelofibrosis?

A

JAK2 kinase mutation in 50% of cases

41
Q

In myelofibrosis, what causes marrow fibrosis?

A

Megakaryocytes produce excess platelet-derived growth factor (PDGF) causing marrow fibrosis

42
Q

What are the clinical features for myelofibrosis?

A

1) splenomegaly 2) leukoerythroblastic smear 3) Increased risk of infection, thrombosis, and bleeding

43
Q

In myelofibrosis what is splenomegaly due to?

A

extramedullary hematopoiesis

44
Q

In myelofibrosis what is the leukoerythroblastic smear?

A

It’s tear-drop RBCs, nucleated RBCs, and immature granulocytes

45
Q

In myelofibrosis what is their an increased risk for?

A

increased risk for infection, thrombosis, and bleeding

Path U2 (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions