Krafts- CML Module Flashcards Preview

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Flashcards in Krafts- CML Module Deck (26):
1

What are chronic myeloproliferative disorders?

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

2

What are the 4 disorders associated w/ CMPDs?

CML
PV
ET
MF

3

CMPDs occur only in________and have a __________course.

adults
LONG

4

What are the common features of CMPDs?

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

5

CMPDs may evolve into.....

Acute leukemias

6

What are the common features associated w/ CML?

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

*Three phases of clinical disease

7

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

are characteristic lab findings of what leukemia?

CML

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?

CML!

**he may also have some lymphadenopathy

9

What are the clinical phases of CML?

SLOW ONSET

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

10

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

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

11

How do you dx CML?

cytogenetics
PCR

12

Polycythemia vera is most commonly associated with what?

HIGH RBC

13

What clinical sx are associated w/ PV?

Thrombosis and hemorrhage

14

What mutation is associated w/ PV?

Jak-2

15

What are the two types of PV?

Primary- Intrinsic myeloid cell problem
Seconadry- d/t increased EPO

16

What are the three main criteria to diagnose PV?

Increased red cell mass
normal O2 sat
splenomegaly

17

How does a mutated JAK 2 cause PV?

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

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?

PV

dizziness, thrombosis, infarction

phlebotomy
mS drugs

19

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

Essential thrombocytothemia

20

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

NO philadelphia chromosome
NO marrow fibrosis
NO other reasons for thrombocytosis

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

21

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

ET

Platelet pheresis
MS drugs
aspirin

22

Chronic myelofibrosis is characterized by _______followed by ____________.

Panmyelosis (many cells of all types)

Marrow fibrosis

23

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

Extramedullary hematopoiesis

24

What causes tear drop cells?

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.

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?

Extramedullary hematopoesis
Severe anemia
Don't have red cells

Chronic myelofibrosis

supportive
MS drugs early on

26

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

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