Myeloproliferative disorders

Question 1

general characteristics of myeloproliferative disorders

Answer 1

increas RBC/WBC/Plts
chronic/acute
terminates in acute leukemia
stem cell or proliferation/differentiation defect
clonal in orgin
middle age-elderly adult
insidious onset (bleeding, infec., thrombosis, anemia)
fibrosis and extramedullary hematopoiesis

Question 2

CML etiology

Answer 2

myeloid cell predominates
idiopathic
environmental: chloramphenicol, radiation, benzene

Question 3

CML clinical presentation

Answer 3

25-60yrold
male predominance
chloromas (increased wbc infiltrate tissues)
green MPO leukemic infiltrates
gout
increased uric acid
increased b12

Question 4

CML laboratory presentation

Answer 4

very increased WBC
diff: shift to left blast, no leukemic hiatus, blast/promyelocytes >10%
increase eos/baso
n/n anemia
increas plt
philadelphia chromo 22;9 (increased tyrosine kinase)

Question 5

LAP stain

Answer 5

leukocyte alkaline phosphatase stain
leukemic granulocytes are non fx
normal LAP: 20-100
leukemic rxn increased
leukemia decreased

Question 6

CML VS. LEUKEMOID RXN

Answer 6

CML: blasts/pros on smear, increased eos/baso, decreased LAP, no toxic granulation/vacuoles, philadelphia chromo.
LEUKEMOID RXN: no blasts/pros on smear, no increased eos/baso, increased LAP, +/= toxic granulation/vacuoles, no philadelphia chromo

Question 7

eosinophilic leukemia

Answer 7

rare
increase. wbc
n/n anemia
decrease. plts
diff: increase. eos, immature eos

Question 8

basophilic leukemia

Answer 8

rarest leukemia
symptoms realated to increase. histamine
increas. wbc
n/n anemia
decreas. plts
diff: increas. basos, immature baso

Question 9

primary myelofibrosis clinical presentation

Answer 9

older adults >50yrs.old
neoplasm of fibroblasts
2* to leukemia, toxins
insidious
splengomegaly
bruising/pallor

Question 10

primary myelofibrosis laboratory presentation

Answer 10

n/n anemia
nRBCs
teardrops
anisocytosis/poikilocytosis
increas. wbc
shift left
increas. eos/baso
decreas. plts

Question 11

leukoerythroblastic picture of primary myelofibrosis

Answer 11

tumor metastisizes to bm
immature cells out of proportion to anemia and wbc count (shift left nRbcs)

Question 12

myelofibrosis therapy

Answer 12

transfusion
antibiotics
splenectomy
death 4-5 yrs (infec., bleeding, thrombosis, cardiac failure)
some convert to ALL

Question 13

CML VS. MYELOPHITHISIC ANEMIA VS. MYELOFIBROSIS

Answer 13

CML: higher wbc, no fibrosis in bm, less rbc poikilocytosis
MYELOPHITHISIC ANEMIA: wbc n/low, tumor cell present in marrow
MYELOFIBROSIS: fibrosis in bm, leukoerythroblastotic picture (immature cells out of proportion to anemia and wbc count), increased wbc, poikilocytosis/anisocytosis

Question 14

polycythemia

Answer 14

rbcs predominate
3 types: primary, secondary, and relative

Question 15

primary polycythemia

Answer 15

polycythemia rubra vera
clonal disease
increase rbc
osler’s disease
may terminate in AML

Question 16

polycythemia rubra vera clincal presentation

Answer 16

insidious
40-60 yrold
viscosity of blood (sludging)
bleeding/ thrombosis
splenomegaly
ruddy complextion

Question 17

polycythemia rubra vera laboratory presentation

Answer 17

bm: erythrocytic hyperplasia, decreased M:E
n/n anemia may become micro/hypo
increased rbc
hgb: >18g/dl
increased wbc/plts
increased LAP

Question 18

polycythemia therapy

Answer 18

no cure
therapeutic phlebotomy
myelosuppressive therapy
survive 3-8 years

Question 19

secondary polycythemia

Answer 19

increase in RBC mass due to another process or disease
(cardiac, pumonary)

Question 20

relative polycythemia

Answer 20

increased hct due to decreased plasma volume (dehydration, vomiting)

Question 21

essential thrombocythemia

Answer 21

plts predominate
unregulated proliferation of megakaryocytes

Question 22

essential thrombocythemia clinical course

Answer 22

middle age adult
bleeding, bruising, thrombosis
abnormal plt fx

Question 23

essential thrombocythemia laboratory presentation

Answer 23

plt count >1million
abnormal apperance and fx
wbc increased
diff: left shift
+/= anemia
increased LDH and uric acid
increased muramidase (enzyme in plts for clotting)

Question 24

essential thrombocythemia therapy

Answer 24

supportive
decrease plt count: plt apheresis (take unit of blood take out bad stuff give back normal stuff)
myelosuppressive drugs
survive 10 yrs

Question 25

reactive thromobocytosis

Answer 25

plt >1 million
transient (acute phase reactants rxt to inflamm)
cause: post surgery, post splenectomy, chronic inflamm., malignancy, severe exercise