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SALMAN HARRISON'S > 99 - Polycythemia Vera > Flashcards

99 - Polycythemia Vera Flashcards

1
Q

What are the two groups of chronic myeloproliferative diseases?

A
  1. Myeloid phenotype

2. Megakaryocyte/erythroid hyperplasia

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2
Q

What are the three myeloid phenotype causing myeloproliferative diseases?

A
  1. CML (myelocytic- BCR-ABL)
  2. CNL (neutrophilic)
  3. CEL (eosinophilic)
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3
Q

What are the three Megakaryocyte/erythroid hyperplasia causing myeloproliferative diseases?

A
  1. PV (polycythemia vera)
  2. ET (essential thrombocytosis)
  3. PMF (primary myelofibrosis)
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4
Q

PV frequency increases with ____, it has a slight ____ tendency, with ____ being more prominent in sporadic cases

A

Age
Familial
Female

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5
Q

In ___ of patients we can find known chromosomal mutations such as ____ deletion or ____

A

30%
13q or 20q
9 trisomy

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6
Q

In ___ we can find ____ mutation where____ replaces ____

A

95%
JAK2
Phenylalanine
Valine

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7
Q

The mutation in JAK2 activates it without ___ dependency leading to an uncontrolled cell productio

A

EPO

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8
Q

Clinical features of PV include: (4)

A

Leukocytosis/Thrombocytosis
Splenomegaly
Thrombus (venous/arterial)
Aquagenic pruritus

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9
Q

Hypervascular erythrocytosis may lead to the following symptoms: (2)

A
  1. Systolic HTN

2. Neurological symptoms (vertigo, headache, visual impairment, TIA, tinnitus)

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10
Q

Thrombocytosis may lead to the following symptoms: (4)

A
  1. Thrombosis (cerebral, GI, hepatic vein)
  2. Acquired vWF
  3. Erythromelalgia
  4. Bleeding disorders (fingers ischemia, etc..)
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11
Q

What is the approach to diagnosing patients with PV?

A

*Increased hct/Hgb?
yes? continue

  • Measure RBC mass
    normal- Relative erythrocytosis
    elevated- continue

*Measure serum EPO levels-
low- PV- confirm with JAK2 mutation
elevated- continue

*Measure arterial O2 saturation
low- DD (COPD, high altitude, AV shunt…)
normal? continue

*Smoker?
no?- measure hemoglobin affinity-
increased- O2 affinity hemoglobinopathy
normal- search for tumors (renal US/CT)
yes?- Measure carboxyhemoglobin
elevated? Smokers polycythemia

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12
Q

In order to confirm absolute erythrocytosis by measuring _____. When unavailable and O2 saturation is normal- check for ____

A

RBC mass

JAK2 mutation

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13
Q

Normal EPO does not disclose ___, but high EPO suggests ____

A

PV

Secondary erythrocytosis

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14
Q

What are the 3 situations leading to microcytic erythrocytosis (check RDW and MCV)?

A
  1. Beta thalassemia (normal RDW)
  2. Hypoxic erythrocytosis ( wide RDW)
  3. PV (wide RDW due to iron deficiency)
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15
Q

___ examination has no value when trying to diagnose PV

A

BM

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16
Q

The lack of ____ does not rule out PV

A

genetic marker

17
Q

What are the two mechanisms leading to PV complications?

A

Hyperviscosity- due to increased amount of cells

Increased cell turnover- increase in cytokines and uric acid

18
Q

_____ infection is more common in PV patients, which may lead to higher frequency of ____

A

H. Pylori

PUD

19
Q

Men with PV should aim for Hgb of ___ and hct of ___, in order to avoid thrombosis.

A

<14

<45%

20
Q

Women with PV should aim for Hgb of ___ and hct of ___, in order to avoid thrombosis.

A

<12

< 42%

21
Q

What are the 3 main treatments of PV?

A
  1. Phlebotomy
  2. Anticoagulation
  3. Ruxolitinib
22
Q

In PV patients, phlebotomy is recommended once every ___ months

A

3

23
Q

If treating PV with anticoagulation, we must avoid ____ when ____ is not under control

A

Salicylates

RBC

24
Q

Ruxolitinib is ____, and is used in PV patients who also suffer from ___ or ____.

A

JAK2 inhibitor
Myelofibrosis
Myeloid metaplasia

25
Q

Asymptomatic _____ (<10) does not require treatment, unless the patient is to be treated with ____. If so, treat with _____

A

Hyperuricemia
Chemotherapy
Allopurinol

26
Q

How would you treat symptomatic splenomegaly?

A
  1. IFN alpha (reduce JAK2 levels)

2. PDE inhibitor/hydroxyurea

27
Q

Why PDE inhibitor is preferred over hydroxyurea when treating PV with symptomatic splenomegaly?

A

PDE inhibitor is not toxic for the BM, and protects from thrombosis

28
Q

If PV patient is resistant to hydroxyurea how will you continue?

A

Ruxolitinib

29
Q

If PV patient is resistant to Ruxolitinib how will you continue?

A

Splenectomy

30
Q

What is PMF?

A

Primary myelofibrosis is a clonal hematopoietic stem cell disorder associated with mutations in JAK2, MPL or CALR and characterized by marrow fibrosis, extramedullary hematopoiesis, and splenomegaly

31
Q

___ are more likely to be diagnosed with PMF, usually in their ___decade of life

A

Men

6th

SALMAN HARRISON'S (163 decks)

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