Flashcards in Myeloproliferative Neoplasms Deck (41)
clonal hematopoietic stem cell diseases with expansion, excessive production and over accumulation of erythrocytes, granulocytes and platelets in some combination; usually aquired
MPNs are predominantly ________ , not acute
disease with overproduction of granulocytes; the cells do differentiate; clonal disorder of HSC (hematopoietic stem cell line)
disease with overproduction of erythrocytes
Disease with increased megakaryopoiesis and thrombocytosis
combination of overproduction of hematopoietic cells and ineffective hematopoiesis with resultant PB cytopenias; fibrosis in BM
characterized by acquired structural chromosomal abnormality: Philadelphia chromosome t(9;22)(q34;q11.2), creates the fusion gene BCR-ABL1
CML Chronic Myelogenous Leukemia
Which one is the derivative chromosome in t(9;22) in CML?
Unique chimeric gene in CML that codes from a protein with enhanced tyrosine kinase activity which leads to increased proliferation and a loss of apoptotic functions
Laboratory findings in CML PB
MK'd leukocytosis, bimodal granulocyte pattern (hallmark), basophilia (Eos can be higher too), Rare nRBC, +/- micromegakaryocytes, increased LD & Uric acid, decreased LAP (stain)
this enzyme is found within secondary granules of neutrophils
Leukocyte ALKALINE phosphatase (LAP)
how do you score the LAP stain? KNOW HOW TO CALCULATE
score 100 cells from 0-4+ and total the scores; 4+ being a cell that has a lot of secondary granules
What is a normal LAP score?
Is the LAP increased or decreased in untreated CML?
Is the LAP increased or decreased in a leukemoid reaction?
normal to increased
Is the LAP increased or decreased in Pregnancy and Polycythemia Vera (PV)?
What do lab findings look like in CML Bone Marrow?
Marked hypercellularity, decreased normoblasts (decreased RBC production), normal to increased megakaryocytes, pseudo-gaucher cells often, and increased reticulin fibrosis
Most patients are diagnosed in the ______ phase of CML, where blasts are <_____%
The ______ phase of CML has a poorer response to therapy, increased anemia, increased clinical symptoms, micromegakaryocytes, etc
Blast crisis is classified as >____% Blasts; worst prognosis
In CML blast crisis extramedually tissues such as the liver and spleen are involved; since the stem cell is affected, blasts can go either way ______ or ______
myeloid or lymphoid
Patients who are Philadelphia Chrom _______ have a worse prognosis than Ph _______ because it progresses more rapidly
How is CML treated?
Synthetic tyrosine kinase inhibitors such as Imatinib (Gleevec), BM transplant, chemo to reduce WBC
This drug competes for the binding site of the BCR-ABL gene and reduces activity of the tyrosine kinase
Chronic Neutrophilic Leukemia is not CML but its close. What is a big difference between the two?
CNL has an increased LAP (not decreased like CML)
What is Polycythemia Vera's mutation?
PV has in increase in what?
Main clinical presentation of PV
Pruritus (but also CNS probs, vision, heart occlusions, ruddy complexion, ulcers, weight loss, splenomegaly)
PV bone marrow manifestations
hypocellular, but M:E is normal
BM iron store are decreased
Megakaryocytes are increased