PV
PV:
What is it?
H and P
overproduction of all three cell lines
more common in males 60yo ish
SPELENOMEGALY IS UNIVERSIAL
what is ET?
H and P
Overprodcution in granulocytes and platelets (esp megakaryocytes)
MDS hallmark
pancytopenia (anemia, bleeding, infection)
what is the defining characteristic of AML
+BCR-ABL
What can CML progress to?
ALL
PV: Labs and DX:
Blood JAK2V617F epo screen positive
Diagnosis of ET
PLT- >450
BM biopsy showing increase of megkaryocye lineage
not meeting WHO criteria for bcr abl+
jak2,calr, mpl mutations
evidence of clonal markers or lack of evidence for reactive thrombocytosis
MDS diganosis and prognosis
bone marrow biopsy and genetic study
prognosis based on % of blasts present and genetics
what is the treatment of AML
H and P and defining charcteristics
affects adults greater than 65yo
auer rods
PV treatment
Phlebotomy to less than 45% in males and females
once daily aspirin (40-100mg)
consider a low-iron diet
clinical features of ET and typical patient
females 50-60
25-33% of patients asymptomatic at time of diganosis
25% have splenomegaly
hemorrhagic: bruising, abnormal bleeding, gi bleeds
thrombotic: ocular migraines, tia, occlusions, pulm htn
MDS H and P
Affects older adults 60+
most are asymptomatic (incidental finding)
typical patient and s/s of CML
57 yo
systemic symptpms
organomegaly
coagulopathy
s/s AML
Fatigue, pallor, abnormal bleeding, malignancies
Leukostasis, CNS symptoms, DIC
ddx of PV
how to treat ET with presentation of thrombosis, major bleeding or >60yo
hydroyurea
MDS treatment
therapy: EPO, g-CSF
cure: stem cell transplant
labs of CML
WBC>150K
RBC usually normal
plt are normal to high
treatment of AML
Aggressive chemo
what two disorders can progress to AML
PV and ET
ALL- luekemia type (b cells)
typical patient and presentation
presents in marrow and blood- very aggressive
almost always younger than 15 yo mostly younger than 3yo
presentation and typical patient
presents as a mass usually in the thymus
more common in males- peak incidence is at 15yo
how to diagnose CML
Bone marrow biopsy and peripheral smear- test both for bcr-abl mutation via PCR
what are favorable and unfavorable makers for prognosis in ALL
Fav: 2-10 y0
low blast count
hyperdiploidy
unfav: less than 2 or older than 10
greater than 100k blasts