hematology - myeloproliferative neoplasms part 2

essential thrombocythemia (ET)

increased megakaryopoiesis and thrombocytosis, counts are greater than 600 and can go as high as greater than 1000

50-60 yo, women around 30

what is the age range for essential thrombocythemia?

one, two

_____ JAK2 mutation is common in ET, _____ are common in PV

essential thrombocythemia (ET)

discovered due to large platelet count on routine lab work or because of vascular occlusion or hemorrhage

microvascular thrombosis

two types of ET, vascular occlusion due to _____ in digits or in large arteries in veins

bleeding

essential thrombocythemia causes _____ in mucous membranes in GI tract, skin, urinary tract, upper respiratory tract

essential thrombocythemia (ET)

diagnosis: need to distinguish from other MPNs, identify mutation to rule out other disorders, bone marrow biopsy helps

essential thrombocythemia (ET)

BM biopsy shows proliferation of mainly the megakaryocyte lineage that are increased and enlarged with hyperlobulated nuclei

peripheral blood ET

thrombocytosis greater than 450 and as high as 5000, giant bizarre platelets that cluster, normochromic erythrocytes

splenic infarct ET

has howell jolly bodies, NRBCs, poikilocytosis

leukocytosis ET

22-40, increased neutrophils with occasional metamyelocytes and myelocytes, basophils and eosinophils normal or slightly increased

bone marrow EV

significany megakaryocyte hypercellularity, they are clustered, bigger, and hyperlobulated

essential thrombocythemia (ET)

treatment: patients experience long survival periods as long as there is no clotting or bleeding, aimed at reducing cellular numbers with medications or plateletpheresis

20

median survival for essential thrombocythemia is _____ years

clotting/bleeding

what is the most common cause of death with essential thrombocythemia?

primary myelofibrosis

least common and most aggressive MPN

5

the average survival for primary myelofibrosis is _____ years

primary myelofibrosis

proliferation of abnormal megakaryocytes and granulocytes in the BM with increase in fibroblasts producing progressive marrow fibrosis

primary myelofibrosis

can form spontaneously or as progression of PV or ET

primary myelofibrosis

major criteria: megakaryocyte proliferation and atypical, age adjusted cellularity, granulocyte proliferation, decrease in erythropoiesis

primary myelofibrosis

minor criteria: anemia not attributed to comorbid condition, leukocytosis, splenomegaly, lactate dehydrogenase above upper limit, leukoerythroblastic

fibroblasts

normal part of bone marrow and produce collagen that provides support structure

primary myelofibrosis

overproduction of collagen that disrupts bone marrow architecture, causes pancytopenia, due to increases in fibroblastic growth factors from platelet granules

over 60

what is the age range for primary myelofibrosis that is equal in males and females?

extramedullary hematopoiesis

in primary myelofibrosis due to release of clonal stem cells into circulation, splenomegaly or hepatomehgaly, accumulates in organs

CD34+

in primary myelofibrosis, number of _____ cells may be 300x normal, this can distinguish it from other MPNs and predicts spleen involvement and risk of conversion to avute leukemia

primary myelofibrosis

can be asymptomatic with slow progression, fever, night sweats, itchy skin, pain in extremities and bones, splenomegaly

intravascular hemolysis

15% of primary myelofibrosis patients have major hemolytic episode _____

peripheral blood primary myelofibrosis

immature granulocytes, NRBCs, teardrop cells and other abnormal shapes, polychromasia; pancytopenia, leukoerythroblastosis, anisocytosis, poikilocytosis

bone marrow primary myelofibrosis

biopsy may be hard to obtain and aspirate may appear normal, the biopsy will show intense fibrosis, hypercellularity, dysplastic changes, dilated sinuses

altered immune response

primary myelofibrosis with antibodies to RBC antigens, nuclear proteins, gamma globulins, phospholipids, organ specific antigens

primary myelofibrosis

at risk for fatal outcomes associated with disease progression (leukemia, thrombohemorrhagic complications, infections)

primary myelofibrosis

treatment: evaluate HSCT eligibility, JAK inhibitors, treat anemia, chemotherapy

leukemic transformation

what is the most common cause of death with primary myelofibrosis?