CML Module - Krafts

Question 1

What is CML?

Answer 1

Chronic Myeloproliferative Leukemia

Question 2

What type of cells are found in the chronic leukemias?

Answer 2

Mature Cells with a WBC Left Shift of Immature Cells

Question 3

Define Chronic Myeloproliferative Disorders

Answer 3

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

Question 4

What are a couple of characteristics of Chronic Myeloproliferative Disorders?

Answer 4

ONLY in Adults
LONG course
- Can potentially live years without treatment

Question 5

What is PV?

Answer 5

Polycythemia Vera

Question 6

What is ET?

Answer 6

Essential Thrombocythemia

Question 7

What is MF?

Answer 7

Myelofibrosis

Question 8

What cells are proliferating most in CML?

Answer 8

Neutrophils

Question 9

What cells are proliferating most in PV?

Answer 9

Red Cells

Question 10

What cells are proliferating most in ET?

Answer 10

Platelets

Question 11

What cells are proliferating most in MF?

Answer 11

Everything

Question 12

What clinical features are associated with Chronic Myeloproliferative Disorders?

Answer 12

Hypercellular Marrow
Large Spleen (no room in marrow for hematopoiesis)
Evolves to Acute Leukemia (more than 20% blasts)
Mutated Tyrosine Kinases

Question 13

What mutation is associated with CML?

Answer 13

Philadelphia chromosome translocation t(9;22)

Question 14

What cell types are associated with CML?

Answer 14

Neutrophilic Leukocytosis and Basophilia

Question 15

What would the lab findings be in CML?

Answer 15

HIGH WBC
Neutrophilia with Left Shift
Basophilia
Low Hemoglobin
Low LAP
Increased Platelet Count (at first)

Question 16

What are the symptoms of CML?

Answer 16

Slow onset
Fever, Fatigue, Night sweats
Abdominal Fullness

Question 17

What is one of the key clinical signs of CML?

Answer 17

Organomegaly
Huge Spleen
Big Liver

Question 18

What are the 3 phases of CML?

Answer 18

Chronic Phase
Accelerated Phase
Blast Crisis

Question 19

Describe the Chronic Phase of CML

Answer 19

Stable counts
Easily controlled
Live for 3-4 years untreated

Question 20

Describe the Accelerated Phase of CML

Answer 20

Unstable counts

Blast crisis within 6-12 months

Question 21

Describe the Blast Crisis of CML

Answer 21

Acute Leukemia
High Mortality
50% of patients will go directly to the Blast Crisis and not enter the Accelerated Phase

Question 22

What are the 3 types of remission in CML?

Answer 22

Hematologic Remission
Cytogenetic Remission
Molecular Remission

Question 23

Describe Hematologic Remission

Answer 23

No splenomegaly

WBC

Question 24

Describe Cytogenetic Remission

Answer 24

No metaphases with t(9;22)

Question 25

Describe Molecular Remission

Answer 25

No BCR/ABL transcripts by PCR

Question 26

What makes the blood sludgy in Polycythemia Vera?

Answer 26

High RBC Count

Question 27

What mutation is associated with Polycythemia Vera?

Answer 27

JAK-2 mutation causing the cells to grow on their own

Question 28

What is the issue in Primary Polycythemia Vera?

Answer 28

Intrinsic Myeloid Cell Problem

Question 29

What is the issue in Secondary Polycythemia Vera?

Answer 29

Due to increased erythropoietin

Question 30

What are the clinical signs of Polycythemia Vera?

Answer 30

Headache, Pruritis, Dizziness Thrombosis Infarction

Question 31

What are the clinical symptoms of Polycythemia Vera?

Answer 31

Organomegaly (spleen and liver) | Plethora (flushing)

Question 32

What is the treatment for Polycythemia Vera?

Answer 32

Phlebotomy | Possibly Myelosuppressive drugs

Question 33

What is the prognosis for Polycythemia Vera?

Answer 33

Median Survival with death occurring from thrombosis or hemorrhage

Question 34

In what patients can Essential Thrombocythemia be seen?

Answer 34

Young Women

Question 35

What is the diagnostic criteria for Essential Thrombocythemia?

Answer 35

Platelet count over 600,000 (usually a million) Hemoglobin of less than 13 Normal RBC Mass None of the following: Philadelphia Chromosome Marrow Fibrosis

Question 36

What are the clinical symptoms of Essential Thrombocythemia?

Answer 36

Bleeding | Thrombosis

Question 37

What are the clinical signs of Essential Throbocythemia?

Answer 37

``` Purpura Bruising Pallor Tachycardia Enlarged Spleen ```

Question 38

What is the treatment of Essential Thrombocythemia?

Answer 38

Platelet Pheresis Myelosuppressive Drugs Aspirin

Question 39

What is the prognosis of Essential Thrombocythemia?

Answer 39

Median Survival with death occurring from thrombosis or hemorrhage

Question 40

What chronic myeloproliferative disorders can have spontaneous leukemia transformation rarely in some patients?

Answer 40

Polycythemia Vera Essential Thrombocythemia Chronic Myelofibrosis

Question 41

What defines Chronic Myelofibrosis?

Answer 41

Panmyelosis (all of the myeloid cell lines are proliferating) with Marrow Fibrosis Extramedullary Hematopoiesis Teardrop RBC

Question 42

What are the clinical symptoms in Chronic Myelofibrosis?

Answer 42

Upper Left Quadrant Fullness | Weakness, Fatigue, Palpitations

Question 43

What are the clinical signs in Chronic Myelofibrosis?

Answer 43

HUGE spleen Pallor Tachycardia

Question 44

What is the treatment of Chronic Myelofibrosis?

Answer 44

Supportive | Myelosuppressive Drugs early on

Question 45

What is the prognosis of Chronic Myelofibrosis?

Answer 45

Median Survival with death from bone marrow failure