Reactive and Neoplastic Myeloid Processes CC Flashcards

1
Q

In order to make a diagnosis of acute leukemia, how many blasts (at a minimum) would you have to see in a peripheral blood (or bone marrow) smear?

A

20%

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2
Q

What is the dominant cell on this slide?

A

Neutrophils

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3
Q

In steptococcall tonsilitis, what is the dominant cell we expect to see?

What about in INfectious mono or whooping cough?

What about cutaneous larva migrans?

A

strep we expect to see neutrophils

In whooping cough or mono = lymphocytosis

cutaenous larva migrans = eosinophils

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4
Q

What test do we order to confirm strep?

for mono?

A

Throat culture for strep

monospot for mono

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5
Q

What do the following lab tests dx or confirm?

JAK2 mutational analysis

Cytogenetics for Philidelphia chromosome

Flow cytometry for TdT+ cells

A

JAK2 mutational analysis = neoplastic condidions

Cytogenetics for Philidelphia chromosome = CML

Flow cytometry for TdT+ cells= ALL

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6
Q

What is the significance of
“toxic change” in the peripheral blood
neutrophils?

A

Indicates the presence of primary granules

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7
Q

What is the difference between primary and secondary granules?

A

Primary granules = purple and seein in BM or in mature neutrophils in reactive conditions

Secondary granules are more pink and are the mature form

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8
Q

When do we see Auer rods?

A

In AML

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9
Q

The patient is a 60-year-old lady with a past medical history significant for hypertension.

Her labs show these concerns:

WBC: 122,000 (normal 4-10,000)

Plates = 550,000 (nomral is 160-370,000)

Neutros = 86,000 (normal is 1,800 to 7,700)

Baso = 1,220 (normal is 0-80)

Negative for infection/medications/ last CBC with normal spleen and lymph nodes; what does this indicate?

A

Chronic myelogenous leukemia

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10
Q

We see hyper/hyocellular BM and

effective or ineffective hematopoesis in CML

A

CML

. Hypercellular bone marrow
with effective hematopoiesis

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11
Q

What is the differnce between effective and ineffective hematopoiesis?

A

efficetive is if precursors are being made in the BM and being released to periphery~ you would see elevated counts, this does not mean cells released are mature

Ineffecitve is when the cells are not released to the periphery thus we will see cytopenias

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12
Q

Leukocytes positive for the
Philadelphia chromosome are often indicitive of

A

Chronic myelogenous leukemia; 9:22 translocation that leads to constituitively active JAK/STAT path

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13
Q

What is the functional defect that is associated with the product of the BCR-ABL
fusion gene?

A

A constitutively activated tyrosine kinase

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14
Q

Clonal myeloproliferative disorder of pluripotent
stem cells
•____ proliferation, _____apoptosis

A

Increased proliferation

decreased apoptosis

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15
Q

CML Hallmarks
– Cytogenetic:
– Molecular:

A

Ph-chromosome

BCR/ABL

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16
Q

Boring statistics on CML

A

7% to 15% of adult leukemias
• Incidence 1.5/105; prevalence 5/105
• 2009 statistics: 5050 new patients, 470 deaths
• Etiology: Irradiation in <5%
Unknown in 95%

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17
Q

What are the three phases in CML?

A

Chronic phases = Ph+ and pts live 3-5 years

Accelerated Phase= cytogenetic changes w/ increasing blasts; live 12-24 mo

Blast phase = increase blasts; live 3-6 months

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18
Q

What type of therapy is recommended for patient with CML?

A

A tryosine kinase inhibitor like Imatinib

19
Q

What are the tryosine kinase inhibitors used to tx CML?

A

Imatinib

Oral = Dasatinib, Nilotinib, bsutinib, Ponatinib

20
Q

If a pt is controlling their CML with a tryosine kinase inhibitor and decides to go off it, what will most likely occur?

A

. The patient will likely progress
to acute leukemia

21
Q

68 yo male comes in with history of abdominal pain;

An upper intestinal endoscopy reveals peptic ulcer disease and an abdominal ultrasound demonstrates hepatic vein thrombosis and splenomegaly.
• A JAK2 mutational analysis is homozygous for
the V617F mutation.

we expect to see decreased or increased EPO?

A

DECREASED erythropoietin

**patient has polycythemia vera

22
Q

The patient is a 22-year-old man who presented to his PCP with weakness and now has blood oozing from his nose and mouth.
• On physical examination, he shows petechiae and ecchymoses over most of his body.
• Laboratory studies show a WBC count of 50,000/mL (reference range: 4,000-10,000/mL), and increases in D-dimers, prothrombin time, and partial thromboplastin time, consistent with disseminated intravascular coagulation (DIC).

HE has bilobed nuclie, chromatin dispersed w/ prominent granules and lots of Auer rods. Dx?

A

Acute myeloid leukemia

23
Q

What cytogenetic abnormality is most
likely associated with the type of AML in our 22 yo pt?

choices are:

t(8;21)

inv(16)

t(15;17)

t(9;11)

A

t15;17

***this is APL and you see auer rods, DIC and is an emergecy

24
Q

What is the functional defect that is associated with the product of the PML-RARa
fusion gene?

A

A block in terminal differentiationdue to retinoic acid receptor disruption

25
Q

What tx would you recommend to patient with a t(15;17) cytogenetic abnormality?

A

This is APL

Tx is All-trans retinoic acid (ATRA)

26
Q

What cytogenetic / molecular abnormalities have a similar prognostic significance to t(15;17), in a patient with AML?

A

t (8;21): AML1/ETO

inv(16): CBF/ MYH11

27
Q

76-year-old woman presenting with
weakness and pancytopenia.
• A bone marrow examination shows a
hypercellular bone marrow with frequent
hypogranular and hypolobated neutrophils,
and small, hypolobated megakaryocytes.

What is the most likely diagnosis based on these findings?

A

Myelodysplastic syndrome

28
Q
  • Which is the most important diagnostic
    criterion in differentiating a myelodysplastic syndrome
    from an acute myeloid leukemia with myelodysplasiaassociated
    changes a patient?
A

The bone marrow blast percentage

29
Q
  • A karyotype is performed and shows
    abnormal findings. What cytogenetic abnormality
    is most likely to be found in this patient that was dx with myelodysplastic syndrome?
A

Monosomy 7

30
Q

What kind of therapy is recommended for patients with myelodysplastic syndrome?

A

A hypomethylating agent, Lenalidomide, Histone Deacetylase inhibitors

you can delier stem cell transplant but IG these patients are older and would have a hard time undergoing transplant

31
Q
A
32
Q

Why do hypOmethlyating agents work for patients with MDS?

A

MDS is HYPERmethlyated DNA thus there is less gene expression because methylation silences gene expression

hypOmethlyation will remove the methly groups from the DNA thus we have gene expression

50% will respond and this lasts 12-18 months

33
Q

50 year old male c/o fatigue
• CBC-D:
– Hb:9 MCV: 100
– Plt: 30,000
– WBC: 80,000 Blasts: 60,000 (60,000/80,000 = 75% blasts!)
• Normal fibrinogen

Flow cytometry shows; CD13 +, CD33 +, HLA-DR +, CD34 +, CD117 +, MPO +

DX?

A

Acute myeloid leukemia

(90% pt we don’t know why they get this, 10% had prior chemo)

34
Q

What is the prognosis for AML based on?

A

age and cytogenetics

35
Q

What are the “Good” cytogeneti associations for AML?

A

t(8;21),

t(15,17),

inv(16),

or with FAB type M3 (28%)

36
Q

What are the ‘poor’ cyotogenetic abnormalities associated with AML?

A

Any patient with adverse karyotypic abnormalities, i.e. -5, -7,
del(5q), abn(3q), complex, or with resistant disease, i.e. >15%
blasts in the bone marrow performed after course 1 In the
absence of favourable karyotypic abnormalities and not FAB
type M3 (20%)

37
Q

What is the recommended Therapy for AML?

A

7 + 3

Ara-C + Daunorubicin for induction

Then HIDAC or Ara-C for Consolidation

38
Q

60 year old gentlemen
• c/o fatigue and night sweats. On exam; fatigue cachexia, bone pain and night sweats
• Labs: Hemoglobin 11 gm/dl
• Exam: Spleen palpable 4 cm at MCL
• Labs: Otherwise unremrkable

Below is his blood smear

DX?

A

Primary myelofibrosis

39
Q

What lab finding would confirm a PMF diagnosis?

A

JAK2 mutational analysis or JAK2 V617F (seen in 50% of cases)

= constantaly on!

40
Q

How often is the JAK2 mutation seen in :

Polycythemia vera?

Essential thrombocythemia?

Primary meylofibrosis?

A

Polycythemia vera? 100%

Essential thrombocythemia? 50%

Primary meylofibrosis? 50%

41
Q

What is staging like for Myelofibrosis?

A

based on Age, hemoglobbin levels, size of spleen and B symptoms

If you have all 4 = 2 year survival

If you ahve none = 9 year survival

42
Q

How do you stage MDS?

A

There are 4 groups and are based on % blasts adn number of cytopenias and chromosomes

Low has 10 year survival

Intermediate 1 and 2 are inbetween

High = 3 month survival

43
Q

How do you stage AML?

A

Good: 60-70% survival

Intermediate: 40-50% survival

Poor cytogenetics= 10-20%