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internal 4th > myeloprolferatibe diseases > Flashcards

myeloprolferatibe diseases Flashcards

1
Q

what effect on the wbc

A

is a cause of neutrophiliaa

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2
Q

what causes neutrophilia

A
  1. infection
  2. inflammation
  3. stress- surgery, infection , trauma, seizures burns
  4. CS use (which can cause lymphopenia)
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3
Q

what can PCV transfromt o

A

secodnary AML

myelofibrosis

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4
Q

PHASES OF PCV

A

latent phase -
proliferative phase - hyperviscosity leading to clots
spent phase - anemia

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5
Q

symptoms of PCV

A

hypertension - too much blood - esp if you prescribe drugs and still not going down

pruritus after bathing

thrombosis

hemorrhages - dodgy plt, so epistaxis, extra menstrual bleeding

very red skin - esp conjunctiva and tongue

HSM - because blood cells stored in spleen

heart problems - as more blood to pump

gout problems- increase turn over

chest pain ,s.ob, when lying down

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6
Q

labs for PCV

A

increase in rbd
increase in hb
hyper uric acid
LOW EPO - unlike in hypoxia

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7
Q

3 main methods of diagnoses PCV

A
  1. low EPO
  2. HIGH HB
  3. JACK 2 MUTATION
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8
Q

hallmark of PCV

A

JACK 2 MUTATION - PCR

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9
Q

TX PCV

A

PHLEBOTMY - doesnt always work

myelosuppressive agents such as hydroxyurea (but increases risk of leukemia) and interferon gama no risk but gives flu like symptoms

last resort is jackave

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10
Q

what age cut off point for tx is considered in PCV

A

> 65 HYDROXYURA
<65 INTERFEON GAMMA

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11
Q

conditions that cause elevated erythrocytosis

A

steroids
high altitudes
dehydration + too much dieurtic ( like pseudo)
renal tumours increase in EPO
COPD

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12
Q

conditions that cause elevated erythrocytosis

A

steroids
high altitudes
dehydration + too much dieurtic ( like pseudo)
renal tumours increase in EPO
COPD

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13
Q

what’s the main issue in PCV

A

blood too thick increase in chance of strokes

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14
Q

what’s the main issue in PCV

A

blood too thick increase in chance of strokes

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15
Q

what cell lines are affceted in pcv

A

ALL But notably RBC

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16
Q

reactive causes for thrombocytosis

A

fe anemia
infection
inflammation
hyposplenism (controls levels of all cell lines)
surgery
malignancy
drugs

17
Q

Mutation of essential thrombocyemia

A

jack mutation but around 50%

CALR
MPL

18
Q

what PLT count in essential thrombocytemia

A

must be 600 on 2 separate counts

19
Q

symptoms of essenital thrombocytemia

A

SPLENOMEGALY
hemorrhage - dodgey platelets
thrmobosis
erythromelalgia - red burnign tichy feet/hands

Dizziness/syncope (secondary to hyper-viscosity).

levido reticularis

20
Q

diagnosis of essential

A

CBC

definitve - bm aspiration hyperplasia of megkarytypes

21
Q

hb in essential

A

normal as just PLT affected

22
Q

tx of essential

A

similar to PCV but no phlebtomy

additional antiaggregrant every day

additional thromboreduction

last resort jackave

23
Q

complication of essentia l

A

acquired von willebrand syndrome

24
Q

causes of increased PLT not to do with reactive

A

essential …
PCV
CML
primary myelofibrosis

25
Q

dx of essential

A

reactive thrombocytosis

26
Q

dx of essential

A

reactive thrombocytosis

27
Q

erythromelalgia>

A

burning extremities accompanied with hot sensation and tingling associated with essential thrombocyt..

28
Q

name the myeloproliferative diseases

A

essential
PCV
CML
MYELOFIBROSIS

29
Q

out of the main myeloproliferative diseases which has the worst prognosis

A

myelofibrosis

30
Q

features of myelofibrosis

A

extramedullary hematopoiesis

MASSIVE SPLENOMEGALY

anemia - bm fibrosis

31
Q

whats the course of myelofibrosis

A

initially there is a hyper proliferative phase (plt)

then panctyponeia - fibrosis

32
Q

diagnosis

A

dry tap on bm aspiration

increase in ALP

33
Q

TX OF MYELOFIBROS

A

straight to jackave (but only 30% works and if it does doesn’t last that long )

ruxolinib

definitve - allogenic

34
Q

labs of myelofibrosis

A

increase uric acid
increase in LDH

35
Q

WHATS THE IMPORTNAT THING TO REMEMBER WITH MYELOFIBROSIS

A

dont do a splenectomy as it increases the progression to AML

36
Q

PATHOGNOMIC FOR MYELOFIBROSIS

A

peripheral blood smear you will see dacrocytes (tear drop shaped associated with extramedullary hemopoeis)

internal 4th (19 decks)

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