Neoplastic Leukocyte Disorders Flashcards
myeloid neoplasms?
myeloproliferative neoplasms, myelodysplastic syndromes, acute myeloid leukemia
myeloproliferative neoplasms?
CML, polycythemia vera, primary myelofibrosis, essential thrombocythemia
qualities of myeloproliferative neoplasms?
hypercellular BM with effective hematopoiesis, clonality causes cytoses. increased PB granulocytes, RBCs, and/or platelets.
CML
BCR-ABL fusion: expansion of stem cells due to constitutively active TK activity.
t (9.22)
leukocytosis with immature myeloid cells, basophilia
3 stages: chronic, accelerated, blasts
CML treatment?
imatinib/gleevec, or SCT
PV
increase RBC, granulocytes, platelets.
JAK 2 mutation: constitutively active.
hyperviscosity: increased RBC mass/Hb, decreased EPO, normal SaO2, increased plasma volume
PV treatment?
phlebotomy, hydroxyurea
Primary myelofibrosis?
rapid BM fibrosis and extrameduallary hematopoiesis (spleen, liver, LNs). MASSIVE splenomegaly. teardrop cells 50% have JAK 2 mut
Primary myelofibrosis tx?
JAK inhibitor
Essential thrombocythemia?
platelets > 450,000. large/giant hypogranular platelets, abnormal megakaryocytes. 50% JAK 2 mut.
Essential thrombocythemia tx?
alkylating agents
myelodysplastic syndromes?
clonal hematopoietic SC disorders w/ cytopenias, dysplasia, and INEFFECTIVE hematopoiesis. increased risk of AML (30% progress). monosomy 7. causes macrocytic anemia. short survival.
myelodysplastic syndrome histo?
dysplastic features, hypercellular BM, ring sideroblasts (mitochondria traps iron). hypogranular and hypolobated PMNs.
myelodysplastic syndrome treatment?
hypomethylating agents (decitabine, azacitidine), or supportive. SCT is curative.
AML?
> 20% blasts, large and uniform w finely dispersed chromatin. auer rods. myelodysplasia.
