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Flashcards in CML Module - Krafts Deck (36)
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1

Chronic leukemias involve:

More mature lymphocytes

2

What things must you know about chronic myeloproliferative disorders?

-Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow [not blocked at a certain stage]
-Four disorders: CML, PV, ET, MF
-Occur only in adults
-Long course (could live a few years without treatment)

3

What are the names of the two chronic leukemias?

These are not benign, they're leukemias!!
1. Chronic myeloproliferative disorders (four)
2. Chronic lymphoproliferative disorders

4

What are the four chronic myeloproliferative disorders?

1. Chronic myeloid leukemia (neutrophils predominating)
2. Polycythemia vera (red cells predominating)
3. Essential thrombocytopenia (not throbocytosis! - megakaryocytic/platelet predominating)
4. Myelofibrosis (everything proliferating same amount, then it becomes fibrinoid)

5

What is unique about chronic myeloproliferative disorders?

-They include mostly mature cells, but can also include some of the lesser developed upper cells in the myeloid lineage

6

What is proliferating most in the myeloproliferative disorders?

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

7

What features are common to all four myeloproliferative disorders?

-Occur only in adults
-Long clinical course
-Inc. WBC with left shift (usually seen in routine CBC)
-Hypercellular marrow
-Big spleen
-May evolve into acute leukemia (almost all of these - as an end stage event, process changes from chronic to acute)
-Mutated tyrosine kinases (CML and other myeloproliferative disorders - mutated thing telling itself to grow all the time)

8

What MUST you know about Chronic Myeloid Leukemia? (EXAM!!)

-Neutrophilic leukocytosis
-Basophilia
-Philadelphia chromosome (EXAM!!)
-Three phases

9

What do you see in slides of CML?

-Abundant mature neutrophils
-Some myelocytes, promyelocytes, blasts
-BASOPHILIA - multiple basophils in a field
-Hypercellular

10

What are the laboratory findings in CML?

-VERY HIGH WBC count (from malignant cells)
-Neutrophilia with left shift (more malignant early cells)
-Basophilia
-Low hemoglobin
-High platelet count (at first) (malignant platelets, normal platelets are decreased in number)
-Dec. LAP (leukocyte alkaloid phosphatase - enzyme present in neutrophils - if bening, LAP should be increased)

11

What chromosomal abnromalities are found in CML?

-Translocation between chromosome 9 and 22
-Chromosome 22 - Philidelphia chromosome - where the action happens
-BCR-ABL hybrid gene --> tyrosine kinase

12

What are the clinical findings (symptoms & signs) in CML?

Symptoms (release of enzymes makes patients sick: fever, night sweats from too many cytokines!):
-Slow onset
-Fever, fatigue, night sweats
-Abdominal fullness
Signs:
-Big spleen
-Big liver

13

What is the chronic phase of CML like?

Most patients present with this, but how they pass through the phases is random in timeline and length
-Stable counts
-Marrow full of cells
-Neutrophil series
-Hemoglobin decreased, platelets up
-Easily controlled (with drugs)
-3-4 years (untreated)

14

What is the accelerated phase of CML?

50% of chronic phase patients go onto accelerated phase.
-Unstable counts
-Blast crisis within 6-12 months
-Things are changing quickly
-Not much you can do for patient
-Patients usually die in this phase or when they go into blast crisis

15

What is the blast crisis phase of CML?

50% of chronic phase patients do onto blast phase. It is extremely difficult to treat and patient likely dies in a month or so.
-Acute leukemia
-More than 20% blasts in blood
-Blasts can be either myeloid or lymphoid!
-High mortality

16

What is CML remission like?

Hematologic Remission
-No splenomegaly
-WBC extremely sensitive

17

What MUST you know about Polycythemia Vera?

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

18

What are the types of Polycythemia Vera? (EXAM!?)

"Polycytemia" = Inc. red cell mass
-Primary (intrinsic myeloid cell problem - low erythropoietin)
-Secondary (due to increased erythropoietin)

19

What are the clinical findings in PV?

Symptoms
-Headache, pruritis, dizziness
-Thrombosis, infarction (clotting and bleeding tendency - something wrong with platelets and the way they interact)
Signs
-Big spleen, liver
-Plethora (flushing of face and neck)

20

What does Polycythemia Vera look like on a slide?

-Red cells are right next to each other/on top of each other
-Bone marrow is hyper cellular
-Erythroid series is predominant

21

What is different about the JAK-2 in PV?

Normal JAK-STAT pathway
--Cell signaling pathway
--Important in many different cell types
JAK-STAT in PV
--Mutated JAK-2; activity increased in PV (JAK activating STAT all the time)
--Cells grow on their own
-Important for diagnosis and drug therapy
-95% of PV have this mutation

22

What is the treatment for PV?

-Phlebotomy
-Maybe myelosuppressive drugs

23

What is the prognosis for PV?

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

24

What things MUST you know about essential thrombocythemia?

-Very high platelet count in blood
-Can occur in young women (still adults) - many other diseases peak in 50s or 60s
-Diagnosis of exclusion
-Thrombosis and hemorrhage

25

What is the diagnostic criteria for ET?

-Need to make sure platelet count is REALLY HIGH
-Platelet count >600,000
-Hgb

26

What are the clinical features of ET?

Symptoms:
-Bleeding
-Thrombosis
Signs
-Purpura, bruising
-Pallor, tachycardia
-Biggish spleen

27

What do you see in the slide (blood) and bone marrow of essential thrombocythemia?

-Lots of platelets here
-Hypercellular bone marrow

28

What is the treatment of ET?

-Platelet pheresis
-Maybe myelosuppressive drugs
-Aspirin

29

What is the prognosis of ET?

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

30

What things MUST you know about Chronic Myelofibrosis?

-Panmyelosis. . . (all myeloid lines are proliferating at once)
-. . .then marrow fibrosis
-Extramedullary hematopoiesis (VERY MASSIVE FOR CM)
-Teardrop red cells