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Heme > Acute leukemia, myeloproliferative, and myelodysplastic syndromes > Flashcards

Acute leukemia, myeloproliferative, and myelodysplastic syndromes Flashcards

1
Q

In acute leukemia, cells are rapidly proliferating but _______

A

undifferentiated/ immature

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2
Q

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

A

ineffective hematopoiesis

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3
Q

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

A

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

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4
Q

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

A

mature

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5
Q

Acute ____ leukemia is more common with aging and in men

A

myelogenous

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6
Q

Acute _____ leukemia is most common in childhood

A

lymphoblastic

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7
Q

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

A

(8;21) translocation

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8
Q

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

A

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

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9
Q

What is the prognosis for CBF- related AML?

A

Generally good, treatment responsive

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10
Q

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

A

Philadelphia chromosome- poor prognosis

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11
Q

Describe the cause and prognosis of APML

A

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

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12
Q

List the main two problems associated with acute leukemia

A

bone marrow failure

organ dysfunction from proliferating leukemia cells

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13
Q

What is leukostasis?

A

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

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14
Q

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

A

Auer rods in AML

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15
Q

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

A

proteins consistent with myeloid lineage- CD13 and CD33

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16
Q

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

A

B cell ALL: CD19, CD22, CD10

T cell ALL: CD7, CD3, CD2

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17
Q

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

A

Favors lymphoid lineage

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

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18
Q

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

A

broad spectrum antibiotics

can back off after getting results of cultures

19
Q

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

A

S phase- DNA synthesis

20
Q

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

A

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

Better survival in ALL

21
Q

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

A

Myeloproliferative

22
Q

List four myeloproliferative diseases

A

CML
polycythemia vera
essential thrombocytosis
primary myelofibrosis

23
Q

CML is defined by the presence of the _____ chromosome

A

Philadelphia

24
Q

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

A

brc-abl

25
Q

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

A

AML and ALL

26
Q

What is seen in the peripheral blood in CML?

A

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
Q

List some presenting symptoms of CML

A

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

28
Q

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

A

Transformation

29
Q

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

A

Imatinib

30
Q

List some common presenting symptoms of polycythemia vera

A 
Headache, fatigue, dizziness, night sweats, visual disturbance, weight loss
Aqugenic pruritis (mast cells)
31
Q

List some physical exam and lab findings in polycythemia vera

A 
splenomegaly
plethora
hepatomegaly
systolic HTN
portal vein thrombosis 
hypercellular marrow
32
Q

Polycythemia vera is associated with a mutation in _____

A

JAK2

- EPO independent hematopoiesis

33
Q

List therapies used in polycythemia vera

A

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

34
Q

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

A

MPL, thrombopoietin receptor, or calreticulin (CALR) gene

35
Q

List therapies for essential thrombocytosis

A

hydroxyurea
aspirin to prevent thrombosis
possibly no treatment

36
Q

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

A

growth signals released by neoplastic hematopoietic stem cells and their progeny

37
Q

What cell morphology is seen with primary myelofibrosis

A

tear drop cells / dacryocytes

38
Q

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

A

JAK2 inhibitor ruxolitnib

Still very poor prognosis

39
Q

Describe myelodysplastic syndrome

A

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
Q

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

A

Hypercellular marrow

decreased production

41
Q

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

A

MDS

42
Q

Describe cell morphologies seen in myelodysplastic syndromes

A

usually anemic/ pancytopenic

neutrophils are hypolobated/ hypogranular
megaloblastic changes in RBCs
Ringed sideroblasts

43
Q

MDS can develop over time into a form of _____

A

AML

Heme (9 decks)

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