Acute leukemia, myeloproliferative, and myelodysplastic syndromes Flashcards Preview

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Flashcards in Acute leukemia, myeloproliferative, and myelodysplastic syndromes Deck (43):
1

In acute leukemia, cells are rapidly proliferating but _______

undifferentiated/ immature

2

Myelodysplastic syndromes are characterized by _______ in which the bone marrow may be hypercellular but peripheral counts are low

ineffective hematopoiesis

3

In myelodysplastic syndromes, cell differentiation is (normal/ abnormal)

abnormal- neutrophils, red cells, platelets, and precursors are morphologically and functionally aberrant

4

In myelodysplastic syndromes, the cells are (mature/immature)

mature

5

Acute ____ leukemia is more common with aging and in men

myelogenous

6

Acute _____ leukemia is most common in childhood

lymphoblastic

7

The form of AML with a _______ translocation is also called core binding factor leukemia because the translocation affects the CBF complex that controls myeloid differentiation

(8;21) translocation

8

Describe the normal role of CBF and how its function is aberrant in (8;21) AML

CBF will bind and attract a collection of transcriptional activators that are required for
transcription of genes necessary for neutrophil maturation

In the 8;21 translocation, CBF-ETO chimeric protein functions as an attractor of transcriptional repressor proteins, and effectively blocks the next step in myeloid differentiation- neutrophils do not differentiate but can divide and proliferate

9

What is the prognosis for CBF- related AML?

Generally good, treatment responsive

10

What is the prognosis for AML or ALL with the 9;22 translocation?

Philadelphia chromosome- poor prognosis

11

Describe the cause and prognosis of APML

15;17 translocation involving the retinoic acid receptor, associated with severe DIC but responds to treatment with ATRA

12

List the main two problems associated with acute leukemia

bone marrow failure
organ dysfunction from proliferating leukemia cells

13

What is leukostasis?

aggregates of leukemic cells cause vascular congestion, occlusion of small vessels, and ischemia that affects multiple organs

14

How can AML be distinguished from ALL on the peripheral blood smear?

Auer rods in AML

15

What CD molecules would be on the surface of a cell in a patient with AML?

proteins consistent with myeloid lineage- CD13 and CD33

16

What CD molecules can be used to distinguish B cell vs T cell ALL?

B cell ALL: CD19, CD22, CD10
T cell ALL: CD7, CD3, CD2

17

What is the significance of expression of TdT in acute leukemia?

Favors lymphoid lineage
TdT is an enzyme necessary for Ig and T cell receptor rearrangement

18

In the setting of acute leukemia, it is standard practice to immediately treat febrile and neutropenic patients with ______

broad spectrum antibiotics
can back off after getting results of cultures

19

The most effective drugs are cycle-specific agents that attack cells during ______

S phase- DNA synthesis

20

True or false: with advances in treatment, AML now has a five year survival rate of nearly 90%

False: 45% for patients younger than 45 and 2% for patients older than 75- still highly lethal

Better survival in ALL

21

________ disorders are characterized by the abnormal proliferation of myeloid stem cells that retain the ability to differentiate and whose progeny function normally

Myeloproliferative

22

List four myeloproliferative diseases

CML
polycythemia vera
essential thrombocytosis
primary myelofibrosis

23

CML is defined by the presence of the _____ chromosome

Philadelphia

24

The Philadelphia chromosome is formed by the 9;22 translocation which causes the formation of the _______ fusion gene

brc-abl

25

The Philadelphia chromosome is also found in some cases of ___ and ____ and portends a poor prognosis in those diseases

AML and ALL

26

What is seen in the peripheral blood in CML?

Granulocytic precursors at all levels of development with a differential similar to normal bone marrow
Basophils and eosinophils
WBC count very high, platelets usually elevated, hematocrit is normal or low

27

List some presenting symptoms of CML

Fever, night sweats, fatigue, anorexia and abdominal pain (enlarged spleen)

28

______ of CML is accompanied by a progressive block in differentiation in which mature WBCs in the marrow are replaced by blasts

Transformation

29

What drug is used to inhibit the tyrosine kinase formed by the bcr-abl fusion gene?

Imatinib

30

List some common presenting symptoms of polycythemia vera

Headache, fatigue, dizziness, night sweats, visual disturbance, weight loss
Aqugenic pruritis (mast cells)

31

List some physical exam and lab findings in polycythemia vera

splenomegaly
plethora
hepatomegaly
systolic HTN
portal vein thrombosis
hypercellular marrow

32

Polycythemia vera is associated with a mutation in _____

JAK2
- EPO independent hematopoiesis

33

List therapies used in polycythemia vera

Phlebotomy (induces Fe deficiency)
aspirin
hydroxyurea
ruxolitnib (inhibits JAK2)

34

Many patients with essential thrombocytosis have JAK2 mutations. Other common mutations include:

MPL, thrombopoietin receptor, or calreticulin (CALR) gene

35

List therapies for essential thrombocytosis

hydroxyurea
aspirin to prevent thrombosis
possibly no treatment

36

In primary myelofibrosis, fibrous tissue infiltrates and replaces normal marrow in response to:

growth signals released by neoplastic hematopoietic stem cells and their progeny

37

What cell morphology is seen with primary myelofibrosis

tear drop cells / dacryocytes

38

What drug can be used in primary myelofibrosis, and how successful is it?

JAK2 inhibitor ruxolitnib
Still very poor prognosis

39

Describe myelodysplastic syndrome

heterogeneous group of clonal hematopoietic stem cell disorders characterized by inability to produce adequate numbers of white cells, red
cells, and/or platelets.

abnormal differentiation
in the progeny of the affected stem cells, causing many of them to undergo apoptosis before reaching maturity--> ineffective hematopoiesis

40

MDS is characterized by a _____ marrow but ____ production of mature blood cells

Hypercellular marrow
decreased production

41

___________ is a potential long term complication of treatment of other cancers

MDS

42

Describe cell morphologies seen in myelodysplastic syndromes

usually anemic/ pancytopenic

neutrophils are hypolobated/ hypogranular
megaloblastic changes in RBCs
Ringed sideroblasts

43

MDS can develop over time into a form of _____

AML