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Heme/Lymph > MDS and AML > Flashcards

MDS and AML Flashcards

1
Q

Cytopenias, dysplasia (one or more
myeloid cell lineages), ineffective
hematopoiesis, and increased risk of
development of AML

A

Myelodysplastic syndromes

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2
Q

Myelodysplastic syndromes are what kind of disorders?

A

Clonal hematopoietic stem cell disorders

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3
Q

Myelodysplastic Syndromes

Enhanced degree of apoptosis contributes to_________
Myeloblasts :

A

cytopenias

Myeloblasts < 20% (PB or BM)

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4
Q

What chromosomal abnormalities do we see in myelodysplastic syndromes in 50% of the cases

A

-5 or del5q, +8, -7 or del7q

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5
Q

What lab findings do we see associated with MDS?

A

 Cytopenias (uni-, bi-, or pancytopenia)
 Leukoerythroblastic reaction
 Dysplastic features
 Hypercellular BM
 Ring sideroblasts
 Increased myeloblasts

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6
Q

What do we see on a blood smear in a patient with MDS?

A

Hypolobulated and hypogranular neutrophils

GIANT platelets and large hypogranular platelets

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7
Q

What do we see on bone marrow aspirate in a patient with Myleodysplastic syndrome

A

dysplastic megakaryocyte (in picture)

dysplastic erythroid precursors

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8
Q

Ring sideroblasts are seen in what disease?

A

Myleodysplastic syndrome

(seen in other conditions as well, so you need to pair this with lab findings and clincal story)

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9
Q

what is wrong in this G banding and what disease is it characteristic of?

A

Monosomy 7

Seen in MDS

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10
Q

How do patients with MDS usually present and what age group?

A

Weaknes, infections, hemorrhage or asymptomatic

In the elderly (50s-80s)

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11
Q

In MDS, what is the median survival?

A

9-29 months

t-MDS is only 4-8 months

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12
Q

What do we worry that MDS will progress to?

What do MDS patients usually die of?

A

Progress to AML in 30% of the cases

Mortality often due to: infection or bleeding as a result of the cytopenias

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13
Q

Therapy for MDS (Three different categories)

A

Supportive: blood products, antibiotics, GFs

Hypomethylating agents: not curative

-Decitabine or Azacitidine

Allogenic stem cell transplant

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14
Q

Define Acute leukemia

A

Neoplastic proliferation of immature
cells (blasts) recapitulating progenitor
cells of the hematopoietic system

(AML or ALL)

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15
Q

What is the difference between Acute and Chronic leukemias

A

Acute = weeks to months with IMMATURE cells

Chronic = months to years with MATURE cells

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16
Q

How common is accute myleodysplatic leukemia and what population do we see it in?

A

3/100,000 per year

seen around 60 yo, 1:1 ratio on male to female

increasing incidence with age: AML = 80-90% of acute adult leukemias

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17
Q

What patient population do we see Acute Lymphoid Leukemia in and how common is it?

A

most common in children; 75% under 18 yo

represents 85% of leukemias in this age group and most common cancer in children

1.4/100,000 a year

M:F is 1.4:1

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18
Q

How do we differentiate ALL and AML besides age and why is it important?

A

Different Tx regimens

baesd on:

 Morphology
 Cytochemistry
 Immunophenotyping
 Genetics

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19
Q

Auer rods and myelodysplasia are present in AML or ALL

A

Auer rods = AML!

myelodyspalisa also seen in AML

Neither present in ALL

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20
Q

Describe the following in AML

Blast size

Chromatin

Nucleoli

Cytoplasm

A

Blasts are large and uniform

Chromatin are finely dispersed

Nuceoli: 1 to 4 often prominent

Cytoplasm: Moderately abundant and granules often present

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21
Q

Describe the following in ALL

Blast size

Chromatin

Nucleoli

Cytoplasm

A

Blast are small to medium; varialbe

Chromatin are course

Nucleoli are absent or 1 to 2 (indisitinct)

Cytoplasm is scant to moderate, no granules

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22
Q

Example of AML HE

A

blasts are large and uniform with 1 to 4 nucleoli, moderately abundant cytoplasm with granules present

(not shown but you do see auer rods 60-70% of the time

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23
Q

HE of ALL

A

Small to medium blasts with coarse chromatin. Absent or indistince nucleoli with scant cytoplasma and no granules in cytoplasm.

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24
Q

What is the purpose of cytochemical stains and what are the two most common?

A

Exploit the presence of intracellular
enzymes that produce a colored product
 Most useful
 Myeloperoxidase (MPO): myeloblasts
 Non-specific esterase (NSE): monocytic
blasts in AMLs with monocytic
differentiation

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25
Q

What’s going on in this HE?

A

This is an MPO staining.. stains AML cells black

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26
Q

What is the purpose of Immunotyping?

A

 Differentiates ALL from AML
 Distinguishes B-ALL from T-ALL
 Identifies subtypes of AML: megakaryocytic,
monocytic, etc.
 Treatment and prognostic groups determined
partly by immunophenotype
 Fingerprint for minimal residual disease (MRD)
assessment

27
Q

Immunotyping in situ is:

Immunotyping of single cells is:

A

in situ is immunohistochemistry, done with tissue and only for one antiG or stain at a time

Of singles cells is Flow Cytometry: done of PB or BM and can do several antiG at a time

28
Q

Key CDs in T linegae

A

CD1a

CD2

CD3

CD4

CD5

CD7 and 8

29
Q

Key CDs for B cell lineage

A

CD 19

CD20

CD22

30
Q

Key generic myeloid CDs

A

CD13, CD15, CD33, CD117, MPO

31
Q

CD41 and CD 61 are associated with

A

megakaryocytes

32
Q

CD 14 is associated with

A

monocytes

33
Q

CD34 is seen in what diseases?

A

Marker of immaturity in AML and ALL

34
Q

TdT is a marker of immaturity seen in

A

ALL

35
Q

CD117 is marker of immaturity seen in:

A

AML

36
Q

CD1a is a marker of immaturity seen in:

A

immature T cells

37
Q

This technique helps classifaction of AML vs ALL associated abnormalities and can define sub-groups within them

A

Cytogenetics (key for biologic and prognostic purposes)

38
Q

AML is a ________ set of disorders with generally poor outcome.

What is overall long term survival?

A

Heterogenous

only 25% survival

39
Q

What type of progenitor cells are invovlved in AML and where do we see it in the body?

A

Myeloid progenitors

mostly in blood and bone marrow but can involve extramedullary sites

40
Q

What type of general symptoms do we see in AML and what is the cause?

A

we see fatigue, weakness, petechiae and infections.. these are a results of the PANCYTOPENIAS

41
Q

What are less common findings of AML

A

organomegaly, lymphadenopathy, infilitration of extramedullary tissues

~ coagulopathy seen in specific variants

42
Q

Key diagnostic criteria for AML

A

Greater then 20% myeloid BLASTS in blood or bone marrow

43
Q

Pathologic features of AML

A

Marrow usually hypercellular
 Variable (but by definition, limited) ability of leukemic clone to mature beyond blast stage
Variable blast morphology, depending on sub-type
 Maturation may be towards any of the myeloid lineages (sometimes more than one)

44
Q

What key hematologic features do we see in AML

A

Severe leukopenia to markedleukocytosis
 Anemia and thrombocytopenia are the norm
 Maturing/mature cells of all myeloid
lineages may be dysplastic

45
Q

WHO classifications of AML

A

AML with recurrent cytogenetic
abnormalities
 AML with myelodysplasia-associated
changes
 AML and MDS, therapy related
 AML not otherwise categorized

46
Q

AML with recurrent cytogenetic abnormalities (“de
novo” AML) have what kind of outcomes?

A

generally favorable

47
Q

Generally reciprocal translocations
 Generally flat incidence rate over different age groups
 Distinctive morphologic features
 Dysplasia of maturing lineages not prominent
 No antecedent myelodysplastic syndrome

A

AML with recurrent cytogenetic abnormalities (“de
novo” AML)

48
Q

In AML with myelodysplastic associated changes, they are likely related to:

A

MDS!!!

*see MDS-type cytogenetic abnormatilies

such as complex karyotypes and losses od chromosomes or part of chromosomes

49
Q

AML with myelodysplasia associated changes;

increasing indicence with ______

prominent _______ dysplasia

______prognosis

A

 Increasing incidence with age
 Prominent multilineage dysplasia
 Poor prognosis

50
Q

Recurrent cytogenetic abnormalities of AML:

list the three favorable ones

A

t(8;21): AML1/ETO
inv(16): CBFb/MYH11
t(15;17): PML/RARa

51
Q

What cytogenetic abnormality is associated with intermediate/unfavorable outcomes?

A

11q23 (MLL) rearrangements

52
Q

What type of AML is associated with t(15;17)

A

Acute promyelocytic leukemia or APL

53
Q

Acute promyelocytic leukemia (APL)

Generally hypo/hypergranular?

 Describe nuclei seen
 Single cells with multiple ______

A

Generally hypergranular
Reniform nuclei
 Single cells with multiple Auer rods (Faggot cells)

54
Q

APL represents ____% of AMLs

A

5-8%

55
Q

What type of AML is this describing?

Usually leukopenic
Frequent DIC at diagnosis causing early morbidity and mortality

A

APL

56
Q

treatement for APL?

A

 Responds to all-trans retinoic acid (ATRA)

57
Q

Role of t(15;17)

A

Produces PML-RARa fusion gene
Retinoic acid important for myeloid maturation;
disruption of receptor produces maturation arrest
at promyelocyte stage

58
Q

How does ATRA work for patients with APL?

A

Pharmacologic doses of all-trans retinoic acid
(ATRA) overcome block and essentially mature
the cells; also quickly corrects the coagulopathy
Responsiveness to ATRA unique to this AML
variant

59
Q

typical markers for Langerhans cell histiocytosis

A

CD1a, langerin

60
Q

What type of disesase do we see these cells in?

A

Birbeck granules (tennis racket apperance)

seen in Langerhan cell histiocytosis

61
Q

What type of mutations are seen with Langerhan cell histocytosis

A

BRAF mutations

62
Q

In Hemophagocytic lymphohistiocytosis /
hemophagocytic syndrome there is very high ___

What primary genetic defect is this associated with?

What about secondary?

A

ferritin

Primary = HLH; defects in perforin gene

Secondary HLH: EVB and lyphomas

63
Q

Hypertrigliceridemia, hypofibrinogenemia,
hemophagocytosis is seen in what condition

A

Hemophagocytic lymphohistiocytosis/hemophgocytic syndrome

Heme/Lymph (10 decks)

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