Krafts- CML Module

Question 1

What are chronic myeloproliferative disorders?

Answer 1

Malignant proliferation of myeloid cells (NOT blasts, maturingn cells) in blood and bone marrow?

Question 2

What are the 4 disorders associated w/ CMPDs?

Answer 2

CML
PV
ET
MF

Question 3

CMPDs occur only in________and have a __________course.

Answer 3

adults

LONG

Question 4

What are the common features of CMPDs?

Answer 4

Hypercellular marrow
Big spleen–d/t extramedullary hematopoeisis
Mutated TRKs
May evolve into

Question 5

CMPDs may evolve into…..

Answer 5

Acute leukemias

Question 6

What are the common features associated w/ CML?

Answer 6

Philadelphia chromosome t(9;22)–>
Neutrophillic leukocytosis
Basophilia

*Three phases of clinical disease

Question 7

Elevated WBC
Neutrophilia w/ left sheft
basophilia
decreased Hb
Increased platelets
decreased LAP

are characteristic lab findings of what leukemia?

Answer 7

CML

Question 8

A 57 y/o M presents to you w/ fever, fatigue, night sweats and says he feels a dragging sensation in his belly. Upon palpation his liver is enlarged.

What’s wrong with him?

Answer 8

CML!

**he may also have some lymphadenopathy

Question 9

What are the clinical phases of CML?

Answer 9

SLOW ONSET

chronic- stable counts, easily controlled
accelerated- unstable counts
blast crisis- lots of blasts

Question 10

What translocation is associated w/ CML and what does it do?

Answer 10

t(9;22)–> BCR-ABL hybrid gene–> codes for a NASTY TRK

Question 11

How do you dx CML?

Answer 11

cytogenetics

PCR

Question 12

Polycythemia vera is most commonly associated with what?

Answer 12

HIGH RBC

Question 13

What clinical sx are associated w/ PV?

Answer 13

Thrombosis and hemorrhage

Question 14

What mutation is associated w/ PV?

Answer 14

Jak-2

Question 15

What are the two types of PV?

Answer 15

Primary- Intrinsic myeloid cell problem

Seconadry- d/t increased EPO

Question 16

What are the three main criteria to diagnose PV?

Answer 16

Increased red cell mass
normal O2 sat
splenomegaly

Question 17

How does a mutated JAK 2 cause PV?

Answer 17

Mut JAK-2>
inability to inhibit JAT-STAT pathway>
excessive cellular growth w/o external signal

Question 18

A pt presents w/ HA, pruritis, a big spleen and plethora. What might they have? What other sxs may you be conerned about? How do you treat them?

Answer 18

PV

dizziness, thrombosis, infarction

phlebotomy
mS drugs

Question 19

What disease has a VERY HIGH platelet count in blood and often occurs in young women?

Answer 19

Essential thrombocytothemia

Question 20

ET is often a diagnosis of exclusion. What lab findings would rule it out.

Answer 20

NO philadelphia chromosome
NO marrow fibrosis
NO other reasons for thrombocytosis

Platelet count > 600,000
Hgb<13 or RBC mass normal

Question 21

A pt presents w/ bleeding, thrombosis, tachycardia, bruising and a big spleen. What might they have? How do you tx them?

Answer 21

ET

Platelet pheresis
MS drugs
aspirin

Question 22

Chronic myelofibrosis is characterized by _______followed by ____________.

Answer 22

Panmyelosis (many cells of all types)

Marrow fibrosis

Question 23

Many CMPDs have this but it is often more so in CMF than others.

Answer 23

Extramedullary hematopoiesis

Question 24

What causes tear drop cells?

Answer 24

In chronic myelofibrosis, tear dropped shaped RBC are formed when RBC can’t move freel through the marrow and spleen d/t the extensive fibrosis.

Question 25

Pt presents w/:
weakness, fatigue, palpitations
Left upper quadrant fullness–>HUGE spleen
pallor and tachycardia

What causes these sxs?
what do they have?
how do you treat them?

Answer 25

Extramedullary hematopoesis
Severe anemia
Don’t have red cells

Chronic myelofibrosis

supportive
MS drugs early on

Question 26

Rank PV, ET, CMF and CML in order of best prognosis.

Answer 26

CML- 3-4 yrs w/ out therapy
CMF-3-5 yr death from marrow failure
ET-508 yr, death from thrombosis or hemorrhage
PV- 9-14, death from thrombosis or hemorrhage

**leukemic transformation can occur in all of these diseases