Chronic Myeloproliferative Disorders Flashcards Preview

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Flashcards in Chronic Myeloproliferative Disorders Deck (26):
1

List the 4 major myeloproliferative disorders

Chronic myelogenous leukemia (CML)
Polycythemia vera
Essential thrombocytosis
Primary myelofibrosis

2

Chronic Myelogenous Leukemia (CML)
Describe the bone marrow cellularity and hematopoiesis

Bone marrow cellularity and hematopoiesis are both increased

3

What is a classic symptom of all myeloproliferative disorders?

Splenomegaly

4

Chronic Myelogenous Leukemia (CML)
What genetic abnormality is present?

t(9;22)
BCR-ABL fusion gene (Philadelphia chr)

5

What is the common pathogenic feature in all myeloproliferative disorders?

Mutated, constitutively activated tyrosine kinases

6

Chronic Myelogenous Leukemia (CML)
Describe what you would find on analysis of peripheral blood

High WBC count
Shift to immaturity (earlier stages like band PMNs, myocytes present)
High basophil counts

7

Chronic Myelogenous Leukemia (CML)
Describe the cellularity of the bone marrow

Cellularity is 100% (no fat cells seen in bone marrow) regardless of age

8

What usually causes a leukemoid reaction?

Infection

9

CML vs Leukemoid Reaction
WBC Count

CML is over 50,000/uL

Leukemoid is usually under 50,000/uL

10

CML vs Leukemoid Reaction
Are the cells mature or immature?

CML - see all stages of maturation present

Leukemoid - Almost all mature

11

CML vs Leukemoid Reaction
What clinical finding might you feel in both (but more commonly in CML)?

Splenomegaly

12

Chronic Myelogenous Leukemia (CML)
Describe the onset and progression of CML

Onset is gradual and progresses slowly

50% of patients enter an accelerated phase with decreased response to treatment, anemia, thrombocytopenia, and may transform to a "blast phase" (an acute leukemia)

13

Chronic Myelogenous Leukemia (CML)
Treatment

Allogeneic bone marrow transplant
IFN-alpha (disliked due to adverse effects)

Imatinib (Gleevac)
Preferred treatment
ABL-specific tyrosine kinase inhibitor
Will normalize WBC counts, improve survival and quality of life

14

Polycythemia Vera
How can it be "relative" or "absolute"?

Relative - decrease in plasma volume lead to an artificial increase in Hct

Absolute - absolute increase in total RBC mass

15

Polycythemia Vera
What mutation commonly causes it?

JAK2 mutation

16

Polycythemia Vera
Pathophysiology

Progenitor cells have a decreased need for EPO (EPO levels are low in the blood)

17

Polycythemia Vera
Clinical and Lab Features

Insidious onset usually in late middle age

RBC counts elevated
Hematocrit may be 60% or higher (normal ~35-50%)

Bone marrow is hypercellular with proliferation of erythroid forms (and granulocytes and megakaryocytes)

18

Polycythemia Vera
Complications

Abnormal blood flow (stasis and vascular distension)
Thrombosis
Bleeding
HTN
Patient may become plethoric (red complexion)

19

Polycythemia Vera
Treatment

Therapeutic phlebotomies (collecting blood and discarding it to lower Hct)

20

Primary Myelofibrosis
What mutations may be present?

JAK2
CALR
MPL

21

Primary Myelofibrosis
What is the primary problem?

Excess collagen deposition (fibrosis) in the bone marrow

22

Primary Myelofibrosis
Describe the progression of the disease

Early phase has hypercellular marrow and not much fibrosis.

Late phase has thick, sclerotic, fibrotic marrow

23

Primary Myelofibrosis
Describe the leukoerythroblastic blood picture

Nucleated RBCs
Tear drop RBCs

24

Primary Myelofibrosis
What stain may be done on the bone marrow to better visualize the fibrosis?

Reticulin stain (type III collagen)

25

Essential Thrombocytosis
What is the primary problem and its major complications?

High platelet counts

Thrombosis or hemorrhage are the main complications

26

Essential Thrombocytosis
Treatment

Aspirin (to prevent thrombosis)

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