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31 Cards in this Set
- Front
- Back
name the most common CLONAL hematologic disorder
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myelodysplastic syndrome
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if blast percentage is greater than 20%, we call that ____ _____.
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acute leukemia
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MDS is primarily a dz of the YOUNG/ELDERLY, with more MEN/WOMEN being affected
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elderly; men
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disorder/cancer that results from chromosome deletion of 5 and 7.
pathogenesis? |
MDS
a mutation in one of the HSC's will give rise to abnormal cells (clonal expansion) --> inc in production of HSC inhibitors that induce T cell mediated cell death--> inc in apoptotic cell death of marrow precursor cells (die before they get out into circulation) |
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what is teh major mechanism of MDS?
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ineffective hematopoiesis
Proliferation of progenitor and early precursor cells is normal – increased (giving a hypercellular marrow), but there is a failure to accumulate mature cells leading to the cytopenias |
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what types of drugs/toxins can predispose to MDS?
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benzene
alkylating agents topoisomerase inhibitors (chemo) |
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what are the clinical manifestations of MDS (3)
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1) anemia -weakness, fatigue
2) thrombocytopenia - bleeding, bruising 3) leukopenia - inc susceptibility to infection |
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what are 3 things you expect to see in red cells in a blood smear of MDS?
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1) basophilic stippling
2) cabot rings 3) Howell jolly: pts with splenic dysfxn |
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what are 2 things you expect to see of white cells on blood smear of pt with MDS
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1) Pseudo-pelger huet: Hyposegmentation of nucleus of neutrophil
2) Stodmeister cell: neutrophil fails to segment at all (so they have single lobe) |
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T or F. micromegakaryocytes are present in MDS
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T.
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identify.
dx? |
micromegakaryocytes
may indicate MDS |
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dimorphic population of rbcs, 5% blasts in marrow, risk of AML is 10% at 2 yrs
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refractory anemia (don't tend to rapidly develop AML)
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type of MDS characterized by ringed accumulation of iron within mitochondria (circling the nucleus)
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refractory anemia with ringed sideroblasts (<5% blasts in marrow, rarely goes to AML)
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type of MDS that occurs primarily in middle-aged women; characterized by macrocytic anemia and normal/increased platelet count (including megakaryocytes with hypolobulated nuclei)
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MDS assoc with isolated deletion of chromosome 5q
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standard tx of MDS
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- EPO, G-CSF, GM-CSF, IL-11
- blood transfusion - deferoxamine (iron chelator) |
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what do you have to be careful about with blood transfusions?
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bewar eof excess iron, so ned to give with iron chelator)
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what is the mechanism of action of vidaza (prodrug of decitabine)
what must you be aware of ? |
allows reactivation of silenced tumor-suppressor genes -- to decrease the proliferation of mutated cell lines
be aware of significant side effects (thrombocytopenia can be difficult to manage) |
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what inclusion is notable here?
dx? |
auer rod (conglomeration of granules in a blast on the way to become mature neutrophils)
acute myelogenous leukemia (AML) |
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AML occurs mostly in CHILDREN/ADULTS.
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adults
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ALL occurs mostly in CHILDREN/ADULTS.
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chilren
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AML type that is assoc with t(15:17)
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M3 acute promyelocytic leukemia (APL)
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AML type that is assoc with inv 16
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M4 Acute myelomonocytic leukemia
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AML type assoc with t(8:21)
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M2 AML with maturation
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AML can present wiht hyperleukocytosis. why is this bad?
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inc risk of CNS involvement...leading to leukostasis primarily affecting lungs and brains
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what are the extramedullary dz's that can occur with AML?
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gum infiltrates (M4/M5)
chloroma (M2) CNS chloroma: the development of multiple, malignant, localized green masses of abnormal cells, usually myeloblasts, especially beneath the periosteum of the skull, spine, and ribs. |
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pt with M3 is esp at risk for what coagulopathy?
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DIC
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what is the most feared complication of acute leukemia
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CNS hemorrhage in a pt with M3 (this is what kills pts early on)
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dx of AML
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first, bone marrow exam + biopsy
then, special stain (Wright-Giemsa, Sudan) & flow cytometry/cytogenetics |
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what is the unique tx of M3 AML (acute promyelocytic leukemia)
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ATRA (all trans retinoic acid) + chemotherapy
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tx of AML.
name some complications of tx |
chemotherapy (complications include prolonged neutropenia/thrombocytopenia)
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when is transplant useful for an AML pt?
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early on after remission (don't wait for relapse)..bc youll have better outcome.
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