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19 Cards in this Set
- Front
- Back
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Mutations in MDS |
if dysregulated or mutated these transcription factors fail to induce differentiation and therefore can cause a prolonged proliferation phase |
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Double hit |
need to increase proliferation and then have a second hit that makes it uncontrolled |
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Acute myeloid leukemias |
immature stages of the myeloid line
rare disease
mostly an adult disease
treatment has not made much progress with the exception of M3 (acute promyelocytic)
dx with molecular techs |
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FAB |
classification proposed in 1976 so that we could all use the same terminology
M1-M7 |
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genetic abrnomalities in AML |
translocation city
progression from MDS into AML leads to worsened prognosis |
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Promyelocyte |
no granules
very basophilic
large nucleus
cant tell on inspection what stage it is in |
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Auer Rods |
megakaryoblast
red sliver lined up granules
pathopneumonic for AML |
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Acute leukemia presentation |
related to cytopenias such as weakness fever bleeding
related to organ involvement; headache abdominal pain bone pain, gum filtration
present with chest pain and WBC with 75,000
rare hematological emergencies, can present with disseminated intravascular coagulation DIC leukostasis (large blasts plugging capillaries in lung and brain) |
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Basic principles for AML treatment |
complete bone marrow exam including morphology, flow and cytogenetics/molecular studies
establish venous access
clinical assessment of patient
will they tolerate treatment, assement of renal function cardiac and coagulation
induction chemo; consildation chemo and bone marrow transplant
supportive care during chemotherapy, use of growth factor support, use of transfusion as inficated |
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Specific Treatment |
cytosine arabinoside and an anthracylcine
induction is the first stage of chemo that is trying to attack the cancer then if remission is achieved continue in the consolidation phase which is a repeat of the cycle
one exception is M3 promyelocytic leukemia |
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Bunches of Auer rods |
very rich in procoagulants
can lead to disseminated intravascular coagulation |
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APL M3 |
promyelocytes rich in auer rods are faggot cells
has a APL/RAR fusion gene product that incolved the retinoic acid receptor
all trans retinoic acid is the treatment and it dissociated the oncogenic protein from the DNA and allowing maturation of the promyelocytes
arsenic can be used to induce remission as well
then chemo to kill the rest |
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Myelodysplastic syndromes |
very common
heterogeneous group of clonal disorders, variable cytopenias, usually presenting in the older patient, tendency to progress to acute myelogenous leukemia
patients usually asymptomatic at presentation but may present with complication secondary to cytopenia or with AML
dyplasia |
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Dysplasia characterisitics in smear |
granulocyte - hypo or hypersegmented, hypo granulated Pelger Huet Cell
red cells - macrocytic, shape changes, basophilic stippling
platelets hypogranular |
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Dysplasia in bone marrow |
Gran: nuclear/cytoplasmic dissynchrony
R: megaloblastic changes in red cells
P: micomegas |
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Myelodisplastic classifications |
number of cell lines
number of blasts
chromosomal findings
ringed sideroblasts |
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Prognosis in MDS |
30% rule
die of acute leukemia die of complications related to cytopenias die of old age |
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Treatment of MDS |
depends on co-morbidities and performance status
range from supportive care chemotherapy an transplant
judicious use of transfusion, treatment of iron overload, use of growth factors, treatment of infection
chemo; decitabine, 5-azacytidine, lenalidomide 5-q- |
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Decitabine and 5-azacytidine |
nucleoside analogs, both are hypomethylating agents that inhibit DNA methyltransferase this may restore the activity of tthese tumor suppressor genes
both are superior to supportive care in certain subgroups of MDS but never a cure |
