Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

IHO Week 4 > CML Module - Krafts > Flashcards

CML Module - Krafts Flashcards

1
Q

Chronic leukemias involve:

A

More mature lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What things must you know about chronic myeloproliferative disorders?

A
  • 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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the names of the two chronic leukemias?

A

These are not benign, they’re leukemias!!

  1. Chronic myeloproliferative disorders (four)
  2. Chronic lymphoproliferative disorders
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the four chronic myeloproliferative disorders?

A
  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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is unique about chronic myeloproliferative disorders?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is proliferating most in the myeloproliferative disorders?

A
  • CML - neutrophils
  • PV - red cells
  • ET - platelets
  • MF - everything!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What features are common to all four myeloproliferative disorders?

A
  • 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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A
  • Neutrophilic leukocytosis
  • Basophilia
  • Philadelphia chromosome (EXAM!!)
  • Three phases
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What do you see in slides of CML?

A
  • Abundant mature neutrophils
  • Some myelocytes, promyelocytes, blasts
  • BASOPHILIA - multiple basophils in a field
  • Hypercellular
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the laboratory findings in CML?

A
  • 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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What chromosomal abnromalities are found in CML?

A
  • Translocation between chromosome 9 and 22
  • Chromosome 22 - Philidelphia chromosome - where the action happens
  • BCR-ABL hybrid gene –> tyrosine kinase
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

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

A 
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the chronic phase of CML like?

A

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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the accelerated phase of CML?

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the blast crisis phase of CML?

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is CML remission like?

A

Hematologic Remission

  • No splenomegaly
  • WBC extremely sensitive
17
Q

What MUST you know about Polycythemia Vera?

A
  • High RBC (makes blood sludgy)
  • Different from secondary polycythemia
  • Thrombosis and hemorrhage
  • Jak-2 mutation
18
Q

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

A

“Polycytemia” = Inc. red cell mass

  • Primary (intrinsic myeloid cell problem - low erythropoietin)
  • Secondary (due to increased erythropoietin)
19
Q

What are the clinical findings in PV?

A 
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
Q

What does Polycythemia Vera look like on a slide?

A
  • Red cells are right next to each other/on top of each other
  • Bone marrow is hyper cellular
  • Erythroid series is predominant
21
Q

What is different about the JAK-2 in PV?

A 
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
Q

What is the treatment for PV?

A
  • Phlebotomy

- Maybe myelosuppressive drugs

23
Q

What is the prognosis for PV?

A
  • Median survival: 9-14 years
  • Death from thrombosis or hemorrhage
  • Leukemic transformation in some patients
24
Q

What things MUST you know about essential thrombocythemia?

A
  • 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
Q

What is the diagnostic criteria for ET?

A
  • Need to make sure platelet count is REALLY HIGH
  • Platelet count >600,000
  • Hgb
26
Q

What are the clinical features of ET?

A 
Symptoms:
-Bleeding
-Thrombosis
Signs
-Purpura, bruising
-Pallor, tachycardia
-Biggish spleen
27
Q

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

A
  • Lots of platelets here

- Hypercellular bone marrow

28
Q

What is the treatment of ET?

A
  • Platelet pheresis
  • Maybe myelosuppressive drugs
  • Aspirin
29
Q

What is the prognosis of ET?

A
  • Median survivial: 5-8 years
  • Death from thrombosis or hemorrhage
  • Leukemic transformation in some patients
30
Q

What things MUST you know about Chronic Myelofibrosis?

A
  • Panmyelosis. . . (all myeloid lines are proliferating at once)
  • . . .then marrow fibrosis
  • Extramedullary hematopoiesis (VERY MASSIVE FOR CM)
  • Teardrop red cells
31
Q

What are the clinical findings in MF?

A 
Symptoms:
-Left upper quadrant fullness
-Weakness, fatigue, palpitations
Signs: 
-Huge spleen
-Pallor, tachycardia
32
Q

What do you see in bone marrow of Chronic myelofibrosis?

A

Hypercellular, swooshy, compact

-LOTS OF RETICULAR FIBERS

33
Q

What does chronic myelofibrosis look like in the blood?

A

Teardrop shaped red cells!

-Cells become transformed when moving through blood stream

34
Q

What is the treatment for MF?

A
  • Supportive

- Maybe myelosuppressive drugs (early on)

35
Q

What is the prognosis for MF?

A
  • Median survival: 3-5 years
  • Death from marrow failure
  • Leukemic transformation in some patients
36
Q

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

A
  • 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”

IHO Week 4 (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions