13 - Myeloproliferative Disorders Flashcards

1
Q

4 major myeloproliferative disorders

A

CML
polycythemia vera
essential thrombocythemia
idiopathic myelofibrosis

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2
Q

are myeloproliferative disorders acquired or congenital?

A

acquired

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3
Q

JAK2 mutation is associated with

A

polycythemia vera, essential thrombocythemia, and myelofibrosis

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4
Q

JAK2 mutation causing myeloproliferative disorder via what mechanism?

A

persistent signaling through spontaneous dimerization in the absence of EPO/TPO

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5
Q

ddx of absolute polycythemia

A

primary - polycythema vera for example. abnormality in erythroid progenitors, grow w/ little to no EPO
secondary - physiologically appropriate (sleep apnea, high altitude, Hb abnormalities) or inappropriate (tumors making EPO, renal dz/transplant, anabolic steroids, cobalt/nickel exposure)

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6
Q

3 congenital polycythemias

A

high O2 affinity hb mutation
2,3 bpg deficiency
congenital methemoglobinemias

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7
Q

clinical presentation of absolute polycythemias

A
HA
thrombosis
hypermetabolic sx - fevers, night sweats, wt loss
pruritis - freq when taking hot bath
plethora - redness in face
splenomegaly
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8
Q

lab dx of polycythemia vera

A
low serum EPO levels (not specific)
JAK2 mutation (95% sensitive)
bone marrow - hypercellular, absence of stainable iron, abnormal karyotype in about 30%
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9
Q

prognosis for polycythemia vera

A

avg survival 10-12 yrs
conversion to spent phase - myeloid metaplasia
converstion to acute leukemia
thrombosis / bleeding

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10
Q

main morbidity assoc w/ essential thrombocythemia

A

thrombotic/bleeding events

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11
Q

diagnostic criteria for essential thrombocythemia

A
platelet count >600k
marked megakaryocytic hyperplasia
abundant platelet clumps
normal red cell mass
no Philadelphia chromosomes or myelofibrosis
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12
Q

3 drugs that can be used to treat essential thrombocythemia

A

hydroxyurea, interferon, anagrelide

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13
Q

features of idiopathic myelofibrosis

A

marrow activity outside bone marrow in areas not active in post fetal life - not result of being crowded out
increased fibrotic activity and reticulin

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14
Q

sx of idiopathic myelofibrosis

A
hypermetabolic
gout
bleeding/thrombosis
anemia
splenomegaly
hepatomegaly
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15
Q

dx of idiopathic myelofibrosis

A

PBS - tear drop RBCs, nucleated RBCs, immature granulocytes
bone marrow - frequent “dry tap”, biopsy is hypercellular/fibrotic
inc number of circulating CD34 cells

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16
Q

tx of idiopathic myelofibrosis

A

stimulators of erythropoiesis, cytotoxic agents (hydroxyurea), splenectomy all options, but have their downsides
thalidomide or revlimid, stem cell transplant

17
Q

prognosis of idiopathic myelofibrosis

A

avg survival 4-5 yrs

common causes of morbidity/mortality include anemia, splenomegaly, acute leukemia, infections/bleeding/thrombosis

18
Q

sx of CML

A
gout
splenomegaly
anemia
bleeding
bone pain
19
Q

findings in CML

A

all stages of granulocyte maturation in PBS
may have elevated platelets
bone marrow hypercellular w/ elevated M:E ratio
Philadelphia chromosome present
dec LAP (leukocyte alkaline phosphatase) score

20
Q

Philadelphia chromosome assoc w/

A

CML