Brainscape's Knowledge GenomeTM

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


See full index

S3B5 Pathology > (MDS/MPN) - Overlap syndromes + Acute Myeloid Leukemia > Flashcards

(MDS/MPN) - Overlap syndromes + Acute Myeloid Leukemia Flashcards

1
Q

Are there any detectable genetic abnormalities for MDS/MPN?

A

yes
but none specific for MDS/MPN

(-)BCR-ABL-1

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

What are the general features seen in MDS/MPN?

A

usually leukocytosis

hypercellular bone marrow

normal/slightly increased blast (<20%)

maturation is present but hematopoiesis effectiveness is variable

organomegaly is common

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

What are the diagnostic criteria for Chronic myelomonocytic leukemia (CMML)?

A
  • persistent monocytosis at least 6 months
  • leukocytosis (common)
  • dyspoiesis present in 1 or more cell lineage
  • <20% blasts in blood & bone marrow
  • No Ph Chromosome or BCR-ABL1 fusion gene
  • No rearrangement of PDGFRA (platelet derived growth factor A), PDGRFAB, FGFR1, or PCM1-JAK2
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

If a patient has all the diagnostic criteria for CMML except that they have >20% blasts, what condition do they have?

A

AML

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

What are the risk factors for developing CMML?

A

older age

male

environmental toxins

radiation

previous chemotherapy

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

What is the clinical presentation of patients with CMML?

A

splenomegaly & hepatomegaly are common

tissue-based leukemic infiltrates may occur

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

What would you expect to seen a peripheral blood smear from a patient with CMML?

A
  • absolute monocytosis
  • +/- absolute leukocytosis, anemia, thrombocytopenia
  • dysplasia (esp. granulocytes)
    • hypolobated neutrophils & hypogranular
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a major differentiating factor between CML & CMML?

A

CML = (+) BCR-ABL1

CMML = (-) BCR-ABL1

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

What would you expect to seen a bone marrow sample from a patient with CMML?

A

dyspoiesis in the aspirate (megakaryocytes)

biopsy with mild/moderate reticulin fibrosis

typically hypercellular (less than CML)

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

What is the prognosis of CMML?

A

may progress to AML

wide spectrum of survival times

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

What is AML & what is the cause?

A

clonal proliferation of immature myeloid cells, usually blasts

caused by acquired mutations of hemopoietic stem cells that impede differentiation & block maturation

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

What tissue is rarely involved with AML?

A

lymph node

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

Is AML more common in adults of children?

A

accounts for 80% of acute leukemias in adults

only 20% in children - except it IS the most common acute leukemia in neonates

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

Does primary or secondary AML have a worse prognosis?

A

AML arising secondary to myelodysplasia or therapy has worse prognosis

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

What type of AML tend to arise de novo?

A

AML with recurrent genetic abnormalities

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

What risk factors are associated with AML?

A

down syndrome

fanconi anemia

bloom syndrome

benzene

alkylating agents

topoisomerase inhibitors

ionizing radiation

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

De novo AML typically has what type of genetic factors associated with it?

What about AML that arises from myelodysplasia or from DNA-damaging drugs?

A
  • primary (de novo)
    • balanced chromosomal translocations
  • secondary
    • deletions or monosomies involving chromosomes 5 & 7
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What clinical signs are often seen in patients with AML?

A
  • nonspecific symptoms
    • anemia, weakness, pallor, malaise, fever, fatigue, bone pain
  • gingival hyperplasia
    • monocytic lineage
  • Disseminated intravascular coagulation (DIC) with acute promyelocytic leukemia
  • organomegaly is uncommon
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

In AML, blasts must comprise >20% of marrow cells except in what cases?

A

AML with specific cytogenetic abnormalities

some erythroleukemias

20
Q

What do the myeloblasts look like in AML?

A

delicate nuclear chromatin

2-4 nucleoli

abundant cytoplasm

fine, azurophilic, peroxidase (+) granules or distinctive peroxidase (+) Auer rods

21
Q

What do the monoblast look like in AML?

A

folded or lobulated nuclei

lack Auer rods

usually express non-specific esterase

usually do not express peroxidase

22
Q

What are the general peripheral blood & marrow features of AML?

A

peripheral counts vary (usually anemia + thrombocytopenia)

marrow cellularity usually increased

blast maturation varies

dysplasia in one or more lineage is common (not required)

23
Q

What is the most common type of childhood AML?

A

AML with t(8;21)(q22;q22); RUNX-1-RUNX1-T1

24
Q

What is the genetic cause of AML with t(8;21)(q22;q22)?

A

Fusion gene of RUNX1 (core binding factor alpha) and RUNX1T1 to form RUNX1-RUNX1T1

25
Q

What are the clinical features of AML with t(8;21)(q22;q22)?

A

predominantly in younger patients

generally some maturation of neutrophil lineage

large & small blasts - frequent Auer rods

good response to chemotherapy

26
Q

What is the genetic cause of AML with inv(16)(p13.1q22)?

A

CBFB-MYH11 fusion gene (core binding factor beta - smooth muscle myosin heavy chain)

27
Q

What are the clinical features of AML with inv(16)(p13.1q22)?

A
  • mostly younger age groups
  • monocytic & granulocytic differnetiation
  • abnormal eosinophil component
    • immature eosinophilic granules are abnormally large - purple & numerous
  • blasts have Auer rods
  • high rate survival
28
Q

What is the genetic mutation associated with Acute Promyelocytic Leukemia with PML-RARA?

A
  • balanced t(15;17)(q22;q22)
    • results in formation PML-RARa fusion gene
    • drives proliferation of promyelocytes
29
Q

What are the two type of Acute Promyelocytic Leukemia with PML-RARA? How are they different?

A
  • microgranular
    • high peripheral blood WBC count - resemble monocytes
    • fine pink granules in cytoplasm
    • usually no discernable Auer rods
    • procoagulants in granules → DIC
  • hypergranular
    • many Auer rods within each cell
30
Q

Why is rapid diagnosis of Acute Promyelocytic Leukemia with PML-RARA so important?

A

patients commonly develop DIC, which is one of the few hematologic emergencies

31
Q

What are the clinical features seen in Acute Promyelocytic Leukemia with PML-RARA?

A
  • typically middle aged adults
  • abnormal promyelocytes predominate
  • strongly myeloperoxidase positive
  • patients commonly develop DIC
32
Q

How is Acute Promyelocytic Leukemia with PML-RARA treated? Prognosis?

A

all-trans retinoic acid (ATRA) & arsenic trioxide

they induce differentiation and maturation of the malignant promyelocytes

Good prognosis

33
Q

What are the clinical features of AML with Myelodysplasia-Related Changes?

A

generally the elderly

severe pancytopenia & multilineage dysplasia

generally poor prognosis

34
Q

What are the criteria for a diagnosis of AML with myelodysplasia-related changes (AML-MRC)?

A
  • AML >20% blasts in blood/BM
    • prior history of MDS or MDS/MPN
    • cytogenic/molecular abnormalities associated with MDS
  • Should NOT have genetic abnormalities that place it into AML with recurrent genetic abnormalities
35
Q

How long after treatment do therapy-related myeloid neoplasms usually develop? How do they present? Prognosis?

A

5-10 yrs

marrow failure & cytopenias

(some will present as overt AML 1-5 yrs after treatment with topoisomerase II inhibitors)

multilineage dysplasia

poor prognosis

36
Q

What is the criteria for a diagnosis of AML, not other wise specified?

A

lack defined genetic abnormality for a specific category

neither therapy-related nor myelodysplasia-related

classification based largely on morphology & cytochemistry

37
Q

Which of the following blood smears is from an acute leukemia & which is from a chronic leukemia?

A
  • Left
    • acute - mainly blasts
  • Right
    • chronic - fewer blasts, mainly more mature cells
38
Q

What is the most common type of leukemia in adults?

A

AML

39
Q

Which cell type, indicative in Acute Promyelocytic Leukemia with PML-RARA, is shown in each of the provided images?

A
40
Q

What type of AML, not otherwise specified is seen in the provided image?

A

AML with minimal differentiation

no myeloid differentiation by morphology or cytochemistry

flow cytometry shows myeloid markers

lasts sometimes resemble lymphoblasts

no granules or Auer rods

41
Q

What type of AML, not otherwise specified is seen in the provided image?

A

Acute myelomonocytic leukemia

proliferation of both neutrophilic and monocytic precursors

large cells with abundant cytoplasm

42
Q

What type of AML, not otherwise specified is seen in the provided image?

A

AML with maturation

similar in morphology to AML

Auer rods & granules often present - some maturation

43
Q

What type of AML, not otherwise specified is seen in the provided image?

A

AML without maturation

high percentage of blasts without evidence maturation

cytochemical stains (myeloperoxidase or sudan black B) are +

flow cytometry shows myeloid markers

blasts with granules +/- Auer rods

44
Q

What type of AML, not otherwise specified is seen in the provided images?

A

Acute/monocytic/monoblastic leukemia

  • 80% or more of bone marrow cells are monocytic lineage
    • acute monoblastic leukemia (>80% monoblasts)
    • acute monocytic leukemia (<80% monoblasts)
45
Q

What clinical symptom is commonly seen in patients with acute monocytic/monoblastic leukemia?

A

extramedullary lesions are common

46
Q

What type of AML, not otherwise specified is seen in the provided image?

A

Acute Erythroid leukemia

extremely rare leukemia

at least 80% of marrow cells are erythroid

erythroblasts often with PAS-positive vacuoles

47
Q

What type of AML, not otherwise specified is seen in the provided image?

A

>50% of blasts are of megakaryocytic lineage

look close, may see some platelets that are pinching off the side

association with mediastinal germ cell tumors observed in young males

S3B5 Pathology (13 decks)

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