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Heme - Amanda > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q
A

ET

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2
Q
A

Myelofibrosis (tear drop cells, monolobed PMNs)

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3
Q

Two most common leukemias

A

CLL (most common) and AML

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4
Q
A

PV (Absence of iron is a typical finding in the marrow at diagnosis. Iron stores have been depleted to sustain the expanded erythrocyte mass.)

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5
Q
A

CML (many different lineages)

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6
Q

Basophilia is common in which myeloproliferative disorders?

A

CML, myelofibrosis, PV

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7
Q

Elevated or low in CML:

Uric acid

LAP

B12

Transcobalamine

A

Uric acid - elevated (tumor lysis syndrome)

LAP - low (myelocytes are non-functional = not producing alk phos)

B12 - elevated

Transcobalamine - elevated (B12 binding protein)

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8
Q

Median duration CML:

Chronic phase

Accelerated phase

Blast phase

A

Chronic phase - 5-6 years

Accelerated phase - 6-9 mo

Blast phase - 3-6 mo

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9
Q

Who discovered Philadelphia chromosome?

A

Janet Rowley

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10
Q

What % of patients with CML have t(9:22)?

A

95%

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11
Q

What % of cells express t(9:22) in person with CML with that cytogenetic abnormality?

A

100% (of RBC, WBC, monocytes, platelets)

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12
Q

Only cure for CML

A

HSCT

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13
Q

Why are busulfan and hydroxyurea not that great?

A

Can’t control CML once it’s in accelerated phase

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14
Q

Targeted tx for CML. MOA?

A

Imatinib - binds in ATP binding pocket of tyrosine kinase, inhibiting it

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15
Q

How long must patient be on imatinib before almost all peripheral blood is back to normal?

A

~1 month!!

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16
Q

More efficacious: IFN or imatinib?

A

Imatinib (much more so)

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17
Q

Resistance to imatinib mediated by what mutation?

A

T-3151

18
Q

Pathway beginning with EPO binding EPO-R

A

Jak-2 activated –> STAT/PI3K/AKT/MAPK –> RBC precursor proliferation and differentiation

19
Q

Jak-2 inhibitors are effective against which diseases?

A

PV, ET, myelofibrosis

20
Q

Jak-2 mutation is most common in which of these: PV, ET, myelofibrosis

A

PT (90-95%)

21
Q

Homozygous Jak-2 mutation indicative of:

A
22
Q

Pt presents with bone pain, portal HTN, muscle wasting, SOB, palpitations. Lab shows thrombocytosis, leukocytosis.

A

Myelofibrosis

23
Q

Two major causes of death for myelofibrosis pts

A

Vascular disease (~35%), acute leukemia (~25%)

24
Q

Two causes of secondary thrombocytosis

A

Refractory thrombocytosis (after BM suppression)

Splenectomy

25
Q

Least common MPD

A

ET

26
Q

Secondary vs ET

A

2ndary = ELEVATED TPO, no splenomegaly, platelet function and morphology is normal, megs are normal

ET = NORMAL TPO, 40% splenomegaly, megs abnormal

27
Q

Aortic and mitral valvular leaflet thickening or vegetations

A
28
Q

3 risk factors for thrombosis

A
  1. Phx
  2. Advanced age
  3. CV risk factors (i.e. smoking)
29
Q

What is Budd-Chiari syndrome and which heme disorder associated with it?

A

Occlusion of hepatic vein

ET

30
Q

Why can ET cause spontaneous abortion/premature deliveries/etc

A

Thrombotic complications causing placental insufficiency

31
Q

Two Rx specifically mentioned for ET

A

Hydroxyurea (ribonucleotide reductase inhibitor)

Anagrelide

32
Q

MOA: Anagrelide

AE?

A

Inhibits differentiation of megs = fewer platelets

CHF, HA, thrombocytopenia

33
Q

Altitude sickness

A

Reactive polycythemia

34
Q

Causes of reactive polycythemia

A

**Anything that would increase EPO

Altitude sickness

Atrial septal defect

Renal hypoxia (due to artery ligation, tumor, hydronephrosis)

Tumor (hypernephroma)

35
Q

Most common symptom of PV

A

HA

36
Q

Blurred vision would be most associated with which MPD?

A

PV (causes distention of retinal vessels)

37
Q

Elevated or low in PV:

LAP

B12

Iron stores

A

LAP - high (hyperfunctional cells)

B12 - high

Iron stores - low

38
Q

Raynaud phenomenon

A

PV

39
Q

TOC for PV

A

Phlebotomy

(myelosuppressive Rx given when platelet/WBC count rises or when pruritic)

40
Q

Cross country runner presents with headache, bleeding, and cyanosis. Dx?

A

Reactive polycythemia due to volume depletion

Heme - Amanda (3 decks)

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