CML Module - Krafts Flashcards by Kayla Schenheit

Chronic leukemias involve:

More mature lymphocytes

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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)

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What are the names of the two chronic leukemias?

These are not benign, they’re leukemias!!

Chronic myeloproliferative disorders (four)
Chronic lymphoproliferative disorders

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What are the four chronic myeloproliferative disorders?

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

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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

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What is proliferating most in the myeloproliferative disorders?

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

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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)

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

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

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What do you see in slides of CML?

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

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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)

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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

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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

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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)

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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

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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

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What is CML remission like?

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Hematologic Remission

No splenomegaly
WBC extremely sensitive

What MUST you know about Polycythemia Vera?

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

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

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“Polycytemia” = Inc. red cell mass

Primary (intrinsic myeloid cell problem - low erythropoietin)
Secondary (due to increased erythropoietin)

What are the clinical findings in PV?

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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)

What does Polycythemia Vera look like on a slide?

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

What is different about the JAK-2 in PV?

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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

What is the treatment for PV?

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Phlebotomy

- Maybe myelosuppressive drugs

What is the prognosis for PV?

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

What things MUST you know about essential thrombocythemia?

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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

What is the diagnostic criteria for ET?

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

What are the clinical features of ET?

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

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

- Lots of platelets here | - Hypercellular bone marrow

What is the treatment of ET?

- Platelet pheresis - Maybe myelosuppressive drugs - Aspirin

What is the prognosis of ET?

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

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

What are the clinical findings in MF?

``` Symptoms: -Left upper quadrant fullness -Weakness, fatigue, palpitations Signs: -Huge spleen -Pallor, tachycardia ```

What do you see in bone marrow of Chronic myelofibrosis?

Hypercellular, swooshy, compact | -LOTS OF RETICULAR FIBERS

What does chronic myelofibrosis look like in the blood?

Teardrop shaped red cells! | -Cells become transformed when moving through blood stream

What is the treatment for MF?

- Supportive | - Maybe myelosuppressive drugs (early on)

What is the prognosis for MF?

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

In a patient with high WBC with a left shift, hyper cellular marrow, and splenomegaly:

- Is it CML? (Ph') - Is it PV? (criteria - headaches, flushing, dizziness, clotting & bleeding, etc.) - Is it MF? (fibrosis - in bone marrow) - Is it ET? (thromobcytosis) - If not, it's "MPD not otherwise specified"

CML Module - Krafts Flashcards

(36 cards)