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IHO Week 4 > CML > Flashcards

CML Flashcards

1
Q

Chronic myeloproliferative disorders: Malignant proliferation of what

A

Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow

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2
Q

Chronic myeloproliferative disorders (4)

A

CML
PV
ET
MF

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3
Q

CMD only happen in what people

A

adults

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4
Q

course of CMD

A

long

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5
Q

What is proliferating most in CML, PV, ET, FM

A

CML - neutrophils
PV - red cells
ET - platelets
MF - everything!

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6
Q

features common to all 4 disorders

A 
Occur only in adults
Long clinical course
INC WBC with left shift
Hypercellular marrow
Big spleen
May evolve into acute leukemia
Mutated tyrosine kinases
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7
Q

CML things you must know

A

Neutrophilic leukocytosis
Basophilia
Philadelphia chromosome
Three phases

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8
Q

if see basophilia, think

A

CML!

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9
Q

philadelphia chromosome –>

A

CML!

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10
Q

Lab findings in CML

A 
INC WBC
neutrophilia with left shift
basophilia
DEC hgb
INC platelet count
DEC LAP
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11
Q

why dec LAP in CML

A

LAP is an enzyme in neutrophils, but in CML they don’t make this enzyme so it goes down
Leukocyte alkaline phosphatase

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12
Q

philadelphia chromosome

A

Normally: 9 has ABL oncogene, 22 has BCR locus

In CML: translocation of ABL onto 22 –> BCR-ABL hybrid gene –> tyrosine kinase

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13
Q

sxs CML

A

Slow onset
Fever, fatigue, night sweats
Abdominal fullness

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14
Q

signs CML

A

Big spleen

Big liver

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15
Q

Phases of CML

A

Chronic
Accelerated phase
Blast crisis

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16
Q

Chronic phase CML

A

Stable counts
Easily controlled
3-4 years (untreated) –> accelerated phase or blast crisis (50/50)

17
Q

Accelerated phase CML

A

Unstable counts

Blast crisis within 6 – 12 months

18
Q

Blast crisis CML

A

Acute leukemia

High mortality

19
Q

Hematologic remission

A

No splenomegaly

WBC

20
Q

Cytogenic remission

A

No metaphases with t(9;22)

21
Q

Molecular remission

A

No BCR/ABL transcripts by PCR

22
Q

Polycythemia vera things you must know

A

High RBC (makes blood sludgy)
Different from secondary polycythemia
Thrombosis and hemorrhage
Jak-2 mutation

23
Q

sxs PV

A

headache, pruritis, dizziness

thrombosis, infarction

24
Q

signs PV

A

big spleen, liver, plethora

25
Q

cytogenetics PV

A

JAK-2

26
Q

Normal JAK-STAT pathway

A

cell signaling pathway

important in many different cell types

27
Q

JAK-STAT in PV

A

Mutated JAK-2: activity increased in PV

Cells grow on their own

28
Q

tx PV

A

Phlebotomy

Maybe myelosuppressive drugs

29
Q

prognosis PV

A

Median survival: 9-14 years
Death from thrombosis or hemorrhage
Leukemic transformation in some patients

30
Q

Essential thrombocythemia

things you must know

A

Very high platelet count in blood
Can occur in young women
Diagnosis of exclusion
Thrombosis and hemorrhage

31
Q

Dx criteria for ET

A

Platelet count >600,000

Hgb

32
Q

signs and sxs ET

A

Bleeding
Thrombosis

Purpura, bruising
Pallor, tachycardia
Biggish spleen

33
Q

tx ET

A

Platelet pheresis
Maybe myelosuppressive drugs
Aspirin

34
Q

Prognosis ET

A

Median survival: 5-8 years
Death from thrombosis or hemorrhage
Leukemic transformation in some patients

35
Q

Chronic myelofibrosis

THINGS you must know

A

Panmyelosis then marrow fibrosis
Extramedullary hematopoiesis
Teardrop red cells

36
Q

Clinical findings MF

A

Symptoms
Left upper quadrant fullness
Weakness, fatigue, palpitations

Signs
Huge spleen
Pallor, tachycardia

37
Q

tx MF

A

Supportive

Maybe myelosuppressive drugs (early on)

38
Q

prognosis MF

A

Median survival: 3-5 years
Death from marrow failure
Leukemic transformation in some patients

IHO Week 4 (15 decks)

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