Myeloproliferative disorders Flashcards
general characteristics of myeloproliferative disorders
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
CML etiology
myeloid cell predominates
idiopathic
environmental: chloramphenicol, radiation, benzene
CML clinical presentation
25-60yrold
male predominance
chloromas (increased wbc infiltrate tissues)
green MPO leukemic infiltrates
gout
increased uric acid
increased b12
CML laboratory presentation
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)
LAP stain
leukocyte alkaline phosphatase stain
leukemic granulocytes are non fx
normal LAP: 20-100
leukemic rxn increased
leukemia decreased
CML VS. LEUKEMOID RXN
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
eosinophilic leukemia
rare
increase. wbc
n/n anemia
decrease. plts
diff: increase. eos, immature eos
basophilic leukemia
rarest leukemia
symptoms realated to increase. histamine
increas. wbc
n/n anemia
decreas. plts
diff: increas. basos, immature baso
primary myelofibrosis clinical presentation
older adults >50yrs.old
neoplasm of fibroblasts
2* to leukemia, toxins
insidious
splengomegaly
bruising/pallor
primary myelofibrosis laboratory presentation
n/n anemia
nRBCs
teardrops
anisocytosis/poikilocytosis
increas. wbc
shift left
increas. eos/baso
decreas. plts
leukoerythroblastic picture of primary myelofibrosis
tumor metastisizes to bm
immature cells out of proportion to anemia and wbc count (shift left nRbcs)
myelofibrosis therapy
transfusion
antibiotics
splenectomy
death 4-5 yrs (infec., bleeding, thrombosis, cardiac failure)
some convert to ALL
CML VS. MYELOPHITHISIC ANEMIA VS. MYELOFIBROSIS
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
polycythemia
rbcs predominate
3 types: primary, secondary, and relative
primary polycythemia
polycythemia rubra vera
clonal disease
increase rbc
osler’s disease
may terminate in AML
polycythemia rubra vera clincal presentation
insidious
40-60 yrold
viscosity of blood (sludging)
bleeding/ thrombosis
splenomegaly
ruddy complextion
polycythemia rubra vera laboratory presentation
bm: erythrocytic hyperplasia, decreased M:E
n/n anemia may become micro/hypo
increased rbc
hgb: >18g/dl
increased wbc/plts
increased LAP
polycythemia therapy
no cure
therapeutic phlebotomy
myelosuppressive therapy
survive 3-8 years
secondary polycythemia
increase in RBC mass due to another process or disease
(cardiac, pumonary)
relative polycythemia
increased hct due to decreased plasma volume (dehydration, vomiting)
essential thrombocythemia
plts predominate
unregulated proliferation of megakaryocytes
essential thrombocythemia clinical course
middle age adult
bleeding, bruising, thrombosis
abnormal plt fx
essential thrombocythemia laboratory presentation
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)
essential thrombocythemia therapy
supportive
decrease plt count: plt apheresis (take unit of blood take out bad stuff give back normal stuff)
myelosuppressive drugs
survive 10 yrs
reactive thromobocytosis
plt >1 million
transient (acute phase reactants rxt to inflamm)
cause: post surgery, post splenectomy, chronic inflamm., malignancy, severe exercise
