Reactive and Neoplastic Myeloid Processes CC Flashcards by Meghan May

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?

20%

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What is the dominant cell on this slide?

Neutrophils

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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?

strep we expect to see neutrophils

In whooping cough or mono = lymphocytosis

cutaenous larva migrans = eosinophils

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What test do we order to confirm strep?

for mono?

Throat culture for strep

monospot for mono

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What do the following lab tests dx or confirm?

JAK2 mutational analysis

Cytogenetics for Philidelphia chromosome

Flow cytometry for TdT+ cells

JAK2 mutational analysis = neoplastic condidions

Cytogenetics for Philidelphia chromosome = CML

Flow cytometry for TdT+ cells= ALL

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

Indicates the presence of primary granules

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What is the difference between primary and secondary granules?

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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When do we see Auer rods?

In AML

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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?

Chronic myelogenous leukemia

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We see hyper/hyocellular BM and

effective or ineffective hematopoesis in CML

CML

. Hypercellular bone marrow
with effective hematopoiesis

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What is the differnce between effective and ineffective hematopoiesis?

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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Leukocytes positive for the
Philadelphia chromosome are often indicitive of

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

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

A constitutively activated tyrosine kinase

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

Increased proliferation

decreased apoptosis

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CML Hallmarks
– Cytogenetic:
– Molecular:

Ph-chromosome

BCR/ABL

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Boring statistics on CML

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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What are the three phases in CML?

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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What type of therapy is recommended for patient with CML?

A tryosine kinase inhibitor like Imatinib

What are the tryosine kinase inhibitors used to tx CML?

Imatinib

Oral = Dasatinib, Nilotinib, bsutinib, Ponatinib

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

. The patient will likely progress
to acute leukemia

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?

DECREASED erythropoietin

**patient has polycythemia vera

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?

Acute myeloid leukemia

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)

t15;17

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

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

A block in terminal differentiationdue to retinoic acid receptor disruption

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

This is APL Tx is All-trans retinoic acid (ATRA)

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

t (8;21): AML1/ETO inv(16): CBF/ MYH11

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?

Myelodysplastic syndrome

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

The bone marrow blast percentage

- 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?

Monosomy 7

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

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

Why do hypOmethlyating agents work for patients with MDS?

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

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?

Acute myeloid leukemia (90% pt we don't know why they get this, 10% had prior chemo)

What is the prognosis for AML based on?

age and cytogenetics

What are the "Good" cytogeneti associations for AML?

t(8;21), t(15,17), inv(16), or with FAB type M3 (28%)

What are the 'poor' cyotogenetic abnormalities associated with AML?

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%)

What is the recommended Therapy for AML?

7 + 3 Ara-C + Daunorubicin for induction Then HIDAC or Ara-C for Consolidation

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?

Primary myelofibrosis

What lab finding would confirm a PMF diagnosis?

JAK2 mutational analysis or JAK2 V617F (seen in 50% of cases) = constantaly on!

How often is the JAK2 mutation seen in : Polycythemia vera? Essential thrombocythemia? Primary meylofibrosis?

Polycythemia vera? 100% Essential thrombocythemia? 50% Primary meylofibrosis? 50%

What is staging like for Myelofibrosis?

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

How do you stage MDS?

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

How do you stage AML?

Good: 60-70% survival Intermediate: 40-50% survival Poor cytogenetics= 10-20%