Flashcards in 277b myeloproliferative neoplasms Deck (18):
JAK2 is involved in hematopoietic growth factor signaling.
Mutations are implicated in myeloproliferative disorders other than CML.
GF --> JAK P self --> downstream signals via STAT
mutation --> active without GF
CML - mutation?
(no JAK2 mutation)
philadelphia chromosome: bcr-abl transformation --> tyrosine kinase activity --> increased cell division, inhibition of apoptosis
CML findings -
Essential thrombocytosis - findings?
increased platelets (little purple dots on smear)
BM have enlarged megakaryocytes that are clustered together
Essential thrombocytosis - what most be excluded?
anything that can cause increased platelet accounts which is more common than ET
post-surgery, splenectomy, infection, etc.
Essential thrombocytosis - clinical presentation?
thrombus --> stroke, PVT, PE
bleeding if ET bad enough
Essential thrombocytosis - mutations
JAK2 or CALR
polycythemia vera - RBC, WBC, Platelets, EPO, mutation
increased x 3 (Hct > 55%) --> suppresses EPO
polycythemia vera - clinical presentation?
gout from increased uric acid
intense itching after a hot shower
erythromelalgia --> severe, burning pain and red color due to episodic blood clot in vessels of extremities
Myelofibrosis - cause?
fibrotic destruction of BM
can arise from ET, PV
Myelofibrosis - smear findings?
leukoerythroblasosis (young WBCs should never see)
tear drop RBCs
Myelofibrosis - BM biopsy?stain?
hypercellularity w/ clustering of abnomral megakaryocytes
stain with reticulin for fibrosis
Myelofibrosis - RBCs? WBCs? platelets?
RBC - decreased --> huge liver and spleen
WBC/Platelets - variable
Myelofibrosis - mutation
JAK2 or CALR
CML - smear findings?
neutrophils, metamyelocytes, basophiles