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IHO Week 4 - WLB > CML Module - Krafts > Flashcards

CML Module - Krafts Flashcards

1
Q

What are the four types of Chronic Myeloproliferative Disorders?

A
  1. Chronic Myeloid Leukemia (neutrophils)
  2. Polycythemia vera (red cells)
  3. Essential thrombocythemia (platelets)
  4. Myelofibrosis (everything)
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2
Q

What are Chronic Myeloproliferative Disorder disorders of?

A

Stem cells!

  • something stimulates stem cells to proliferate, but there is no differentiating block
  • malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood and/or bone marrow
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3
Q

What age group do Chronic Myeloproliferative Disorders occur in?

A

ONLY ADULTS (20+)

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4
Q

What is the course of Chronic Myeloproliferative Disorders?

A
  • Long course
    • if you don’t treat the patient they will still live for a few years then die
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5
Q

What are 7 common features of all four Chronic Myeloproliferative Disorders?

A
  1. Occur only in adults
  2. Long clinical course
  3. Increased WBC count with left shift
  4. Hypercellular bone marrow
  5. Splenomegaly
  6. Mutated tyrosine kinases
  7. May evolve into acute leukemia
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6
Q

What are the typical lab findings in CML?

A
  • High WBC count
  • Neutrophilia with left shift
  • Basophilia
  • Low hemoglobin
  • Increased platelet count (at first)
  • Decreased Leukocyte Alkaline Phosphatase (LAP)
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7
Q

What abnormality is seen on a cytogenetic assay in CML?

A
  • chromosomal translocation between chromosome #9 and chromosome #22
    • forms BCR-ABL hybrid gene
    • “Philadelphia chromosome” = chroms 22
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8
Q

What does the bone marrow look like in CML?

A
  • hypercellular
  • pan-myeloid hyperplasia
    • all the myeloid cells are increased –neutrophils and precursors, red cell precursors, megakaryocytes
    • neutrophils and precursors make up the bulk of the cells
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9
Q

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

A
  • Slow onset
  • Fever, fatigue, night sweats
  • Abdominal fullness
    • Big spleen
    • Big liver
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10
Q

Name the three phases of CML.

A
  • Chronic phase
  • Accelerated phase
  • Blast crisis
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11
Q

What signifies the Chronic Phase of CML?

A
  • High but stable number of neutrophils and precursors.
  • Stable hemoglobin and platelet count.
  • Easily controlled by therapy.
    • With traditional treatment (not imatinib), usually lasts 3-4 years
    • followed by accelerated phase and/or blast crisis.
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12
Q

What signifies the Accelerated Phase of CML?

A
  • Characterized by a change in the patient’s previously stable state → unstable
    • Usually see increasing leukocytosis, decreasing hemoglobin and platelet count.
  • May terminate in this stage, or may progress to blast crisis.
  • Usually fatal within several (6-12) months
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13
Q

What signifies the Blast Crisis Phase of CML?

A
  • Characterized by a marked increase in blasts (myeloblasts or lymphoblasts).
    • Acute leukemia
  • Usually fatal within a few weeks or months.
    • hardest phase to treat
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14
Q

What are the three types of “Remission” in CML?

A
  • Hematologic remision
    • no splenomegaly
    • WBC<10,000, normal morphology (may still be malignant cells around)
    • Normal hemoglobin & platelet count
  • Cytogenetic remission
    • No metaphases with t(9;22)
  • ***Molecular remission
    • No BCR/ABL transcrips by PCR
    • really sensitive
    • done on blood and/or bone marrow
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15
Q

What is Polycythemia vera (PV) characterized by?

A

panmyelosis with an erythroid predominance

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16
Q

What does “Polycythemia” mean?

A

increased red cell mass

17
Q

What is Primary Polycythemia?

A

Increase in red blood cells caused by an intrinsic abnormality of myeloid cells

(no ↑ in erythropoietin)

18
Q

What is Secondary Polycythemia?

A

increase in red blood cells caused by ↑ secretion of erythropoietin, which may be:

  • appropriate (e.g., high-altitude living) or
  • inappropriate (e.g., a paraneoplastic syndrome related to a solid tumor).
19
Q

What does the bone marrow look like in Polycythemia Vera?

A
  • hypercellular
    • with a pan-myeloid hyperplasia
  • Red cells make up the bulk of the myeloid cells in the marrow
20
Q

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

A
  • Headache, dizziness
    • caused by blood sludging in vessels
  • Pruritis
    • caused by basophils
  • Thrombosis, infarction
    • due to change in red cell mass and how they interact with the endothelial cells
  • Big spleen & liver
  • Plethora (flushing of face and/or neck)
21
Q

What pathway is overactive in Polycythemia Vera? Why?

A
  • JAK-STAT pathway = overactive
    • due to mutated JAK-2 → increased activity
    • cells grow on their own, think that it is always getting signals to grow, but it is not
    • inhibitory domain of JAK does not work properly
22
Q

What is the treatment for Polycythemia Vera?

A
  • Phlebotomy
  • Maybe myelosuppressive drugs
23
Q

What is the prognosis for patients with Polycythemia Vera?

A
  • Median survival = 9-14 years
  • Death from thrombosis or hemorrhage
  • Acute leukemic transformation in some patients
24
Q

What is Essential Thrombocythemia characterized by?

A

panmyelosis, with a megakaryocytic predominance

25
Q

What are the diagnostic criteria for Essential Thrombocythemia?

A

***DIAGNOSIS OF EXCLUSION***

  • Platelet count >600,000 (usually over 1 mil)
  • Hemoglobin <13 or RBC mass normal
  • No Philadelphia chromosome
  • No marrow fibrosis
  • No other reason for thrombocytosis
26
Q

What are the clinical features (signs & symptoms) of Essential Thrombocythemia?

A
  • Bleeding
  • Thrombosis (myocardial infarction, stroke, and deep venous thrombosis)
  • Purpura, bruising
  • Pallor, tachycardia
  • Biggish spleen (mild splenomegaly)

(Can occur in young women 20-30s)

27
Q

What does the blood/bone marrow look like in Essential Thrombocythemia?

A
  • BLOOD = ton of abnormal platelets
    • large and/or hypogranular
  • MARROW = increased cellularity
    • tons of megakaryocytes, which are often described as being “back-to-back.”
28
Q

What is the treatment for Essential Thrombocythemia?

A
  • Platelet pheresis
  • Maybe myelosuppressive drugs
  • Aspirin (prevent thrombosis)
29
Q

What is the prognosis for a patient with Essential Thrombocythemia?

A
  • Median survival = 5-8 years
  • Death from thrombosis or hemorrhage
  • Acute leukemic transformation in some patients
30
Q

What is Chronic Myelofibrosis characterized by?

A
  • panmyelosis
  • bone marrow fibrosis
  • massive extramedullary hematopoiesis
31
Q

What are the clinical findings in Chronic Myelofibrosis?

A
  • splenomegaly
    • left upper quadrant pain and fullness
    • epigastric pressure
  • anemia (weakness, fatigue, and palpitations)
    • pallor
    • tachycardia
32
Q

What does the blood/bone marrow look like in Chronic Myelofibrosis?

A
  • BLOOD:
    • leukoerythroblastosis (looks like leukemia but it isn’t)
    • teardrop red cells
    • weird looking platelets (large and hypogranular)
  • MARROW:
    • hypercellular, with a pan-myeloid hyperplasia
    • fibrotic, with very few hematopoietic cells left
33
Q

What is the treatment for Chronic Myelofibrosis?

A
  • Supportive
  • Maybe myelosuppressive drugs (early on)
34
Q

What is the prognosis for a patient with Chronic Myelofibrosis?

A
  • Median survival = 3-5 years
  • Death due to bone marrow failure
  • Acute leukemic transformation in some patients
35
Q

What could a patient with a high WBC with a left shift, hypercellular bone marrow, and splenomegaly have?

A
  • CML? (philadelphia chromosome)
  • Polycythemia Vera? (exclusion criteria)
  • Myelofibrosis? (fibrosis)
  • Essential Thrombocythemia? (thrombocytosis)

***IF NOT, “Myeloproliferative Disease not otherwise specified”

IHO Week 4 - WLB (13 decks)

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