Path/Myeloproliferatives Flashcards
What is the big problem with myeloproliferative diseases?
Chronic proliferation of clones that differentiate to circulating blood cells
What cell dysfunctions occur in CML?
High WBC
All stages of granulocytes in blood
What is the main difference between CML and CMML?
CMML shows abnormal monocyte lineages also
PV defect?
Erythroid lineage overactive –> elevated RBC count
ET and myelofibrosis main defect
Mutant megs cause elevated platelet count
Factors that help you distinguish infection from myeloproliferative disease?
Left shift Toxic granulation Symptoms Check smear WBC - myeloid bulge (more myelocytes than metamyelocytes )
CML
t(9;22) BCR-ABL1 Tyr kinase overactivity
carcinogen exposure-related
30-60 yo
Imatinib (if no resp use Dadat- and nilotinib)
Use PCR Low LAP (nonfunctioning lymphocytes)
Hypercellular
No blasts
Basophilia in PB
PV what is the predominant cause?
JAK2V617F mutation
Mastocytosis cause
C-KIT or PDGF-RA activation
Mastocytosis immunophenotype
Tryptase!
CD117+
CD25+ usually
Mastocytosis treatment
Imatinib
Myelofibrosis cause
Jak2 mutation on chrmo 9
Myelofibrosis pathogenesis
Abnormal megs make cytokines –> collagen deposition from activated fibroblasts
JAK2 is mutated in these myeloproliferative diseases
Myelofibrosis, ET, PV
90% of cases of this disease involve JAK2 mutations
PV