myeloprolferatibe diseases Flashcards
what effect on the wbc
is a cause of neutrophiliaa
what causes neutrophilia
- infection
- inflammation
- stress- surgery, infection , trauma, seizures burns
- CS use (which can cause lymphopenia)
what can PCV transfromt o
secodnary AML
myelofibrosis
PHASES OF PCV
latent phase -
proliferative phase - hyperviscosity leading to clots
spent phase - anemia
symptoms of PCV
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
labs for PCV
increase in rbd
increase in hb
hyper uric acid
LOW EPO - unlike in hypoxia
3 main methods of diagnoses PCV
- low EPO
- HIGH HB
- JACK 2 MUTATION
hallmark of PCV
JACK 2 MUTATION - PCR
TX PCV
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
what age cut off point for tx is considered in PCV
> 65 HYDROXYURA
<65 INTERFEON GAMMA
conditions that cause elevated erythrocytosis
steroids
high altitudes
dehydration + too much dieurtic ( like pseudo)
renal tumours increase in EPO
COPD
conditions that cause elevated erythrocytosis
steroids
high altitudes
dehydration + too much dieurtic ( like pseudo)
renal tumours increase in EPO
COPD
what’s the main issue in PCV
blood too thick increase in chance of strokes
what’s the main issue in PCV
blood too thick increase in chance of strokes
what cell lines are affceted in pcv
ALL But notably RBC
reactive causes for thrombocytosis
fe anemia
infection
inflammation
hyposplenism (controls levels of all cell lines)
surgery
malignancy
drugs
Mutation of essential thrombocyemia
jack mutation but around 50%
CALR
MPL
what PLT count in essential thrombocytemia
must be 600 on 2 separate counts
symptoms of essenital thrombocytemia
SPLENOMEGALY
hemorrhage - dodgey platelets
thrmobosis
erythromelalgia - red burnign tichy feet/hands
Dizziness/syncope (secondary to hyper-viscosity).
levido reticularis
diagnosis of essential
CBC
definitve - bm aspiration hyperplasia of megkarytypes
hb in essential
normal as just PLT affected
tx of essential
similar to PCV but no phlebtomy
additional antiaggregrant every day
additional thromboreduction
last resort jackave
complication of essentia l
acquired von willebrand syndrome
causes of increased PLT not to do with reactive
essential …
PCV
CML
primary myelofibrosis
