MDS, MPSs - Hubbard Flashcards

1
Q

MDS are disorders of what?

Characterized by what?

A

Pluripotent stem cell

Ineffective hematopoiesis

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2
Q

Pts w/HA, dizziness, tinnitus, blurred vision, and fatigue most likely have what?

What other common symptom?

A

PV

Hyperviscosity

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4
Q

What subset of pts may benefit from androgen therapy for MDS?

A

Hypoplastic marrow pts

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6
Q

What cytogenetics give a poor prognosis in MDS?

A

Monosomy 7
HypOdiploidy
multiple abn.

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7
Q

treatment of MDS?

A

Supportive care and aggressive tx of infections

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8
Q

What is the only curative tx for PM?

A

Allogenic stem cell transplant

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10
Q

What is the tx for MDS in high intensity therapy?

A

AML style, not as effective as de novo AML

HSC transplant if under 60 and has HLA matched sibling donor

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12
Q

What is the pathogenesis of myelofibrosis?

A

Increased reticulin deposition in the marrow

2ndary to increased PDGF

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13
Q

What labs are abnormal in MDS?

A

Inc LDH
Iron overload
Inc ferritin

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16
Q

What are the adverse prognosis features of MDS?

A
Marrow blasts > 5%
Platelets < 100,000
Hb < 10
Neutrophils < 2500
Age > 60
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17
Q

What mutation is present 45-65% of PM?

A

JAK2

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18
Q

What is the most common cause of death in PM?

A

Overwhelming infection

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21
Q

When is EPO ineffective in pts with MDS?

A

EPO > 500

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22
Q

Describe the following in PV:

Hct?

EPO?

A

High

Low

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24
Q

What is the tx for low/intermediate intensity therapy for MDS?

A

Hypomethylating agents like azacitidine and decitabine

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25
Q

What is the most common complication of PV?

Treatment?

A

Thrombosis

Phlebotomy

26
Q

What MDS type has the lowest risk of conversion to AML (10-15%)?

A

Refractory Anemia w/Ringed Sideroblasts

27
Q

Leukoerythroblastic anemia, poikilocytosis and splenomegaly are characteristic of what?

What classic clinical feature?

A

Primary myelofibrosis

Dry tap on bone marrow aspirate

28
Q

What is the Etiology of MDS?

A
Chemo
Tx for breast cancer, HL, NHL, Acute leukemia
Anthracyclines, alkylating agents
Radiation
Oil and shipyard workers
29
Q

What new drug is approved for tx of intermediate and high risk myelofibrosis?

A

Ruxolitinib

30
Q

What is the disease course for PM?

A

Chronic but progressive

32
Q

Pts w/PM and splenomegaly, what are the 3 treatments in progressive intensity?

A

Hydroxyurea
Radiation
Splenectomy

34
Q

Describe the general MDS clinical picture

A

Pancytopenia w/hyperplastic marrow

37
Q

What cytogenetics give a favorable prognosis in MDS?

What drug has a good effect in this type?

A

5q-

Lenalidomide (angiogenesis inhibitor)

38
Q

If after 6 months of giving B6 to a pt w/RARS there is no improvement, what does it mean?

A

B6 resistant RARS

40
Q

Cytogenetic of MDS?

A

Inv16
Deletion of 5 or 7
Trisomy 8

46
Q

Pts with RARS have a deficiency in what?

A

Pyridoxine (B6)