Hematology (Mds & Aml)

Front 1

name the most common CLONAL hematologic disorder

Back 1

myelodysplastic syndrome

Front 2

if blast percentage is greater than 20%, we call that ____ _____.

Back 2

acute leukemia

Front 3

MDS is primarily a dz of the YOUNG/ELDERLY, with more MEN/WOMEN being affected

Back 3

elderly; men

Front 4

disorder/cancer that results from chromosome deletion of 5 and 7.

pathogenesis?

Back 4

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)

Front 5

what is teh major mechanism of MDS?

Back 5

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

Front 6

what types of drugs/toxins can predispose to MDS?

Back 6

benzene
alkylating agents
topoisomerase inhibitors (chemo)

Front 7

what are the clinical manifestations of MDS (3)

Back 7

1) anemia -weakness, fatigue
2) thrombocytopenia - bleeding, bruising
3) leukopenia - inc susceptibility to infection

Front 8

what are 3 things you expect to see in red cells in a blood smear of MDS?

Back 8

1) basophilic stippling
2) cabot rings
3) Howell jolly: pts with splenic dysfxn

Front 9

what are 2 things you expect to see of white cells on blood smear of pt with MDS

Back 9

1) Pseudo-pelger huet: Hyposegmentation of nucleus of neutrophil
2) Stodmeister cell: neutrophil fails to segment at all (so they have single lobe)

Front 10

T or F. micromegakaryocytes are present in MDS

Back 10

T.

Front 11

identify.

dx?

Back 11

micromegakaryocytes

may indicate MDS

Front 12

dimorphic population of rbcs, 5% blasts in marrow, risk of AML is 10% at 2 yrs

Back 12

refractory anemia (don't tend to rapidly develop AML)

Front 13

type of MDS characterized by ringed accumulation of iron within mitochondria (circling the nucleus)

Back 13

refractory anemia with ringed sideroblasts (<5% blasts in marrow, rarely goes to AML)

Front 14

type of MDS that occurs primarily in middle-aged women; characterized by macrocytic anemia and normal/increased platelet count (including megakaryocytes with hypolobulated nuclei)

Back 14

MDS assoc with isolated deletion of chromosome 5q

Front 15

standard tx of MDS

Back 15

- EPO, G-CSF, GM-CSF, IL-11
- blood transfusion
- deferoxamine (iron chelator)

Front 16

what do you have to be careful about with blood transfusions?

Back 16

bewar eof excess iron, so ned to give with iron chelator)

Front 17

what is the mechanism of action of vidaza (prodrug of decitabine)

what must you be aware of ?

Back 17

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)

Front 18

what inclusion is notable here?

dx?

Back 18

auer rod (conglomeration of granules in a blast on the way to become mature neutrophils)

acute myelogenous leukemia (AML)

Front 19

AML occurs mostly in CHILDREN/ADULTS.

Back 19

adults

Front 20

ALL occurs mostly in CHILDREN/ADULTS.

Back 20

chilren

Front 21

AML type that is assoc with t(15:17)

Back 21

M3 acute promyelocytic leukemia (APL)

Front 22

AML type that is assoc with inv 16

Back 22

M4 Acute myelomonocytic leukemia

Front 23

AML type assoc with t(8:21)

Back 23

M2 AML with maturation

Front 24

AML can present wiht hyperleukocytosis. why is this bad?

Back 24

inc risk of CNS involvement...leading to leukostasis primarily affecting lungs and brains

Front 25

what are the extramedullary dz's that can occur with AML?

Back 25

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.

Front 26

pt with M3 is esp at risk for what coagulopathy?

Back 26

DIC

Front 27

what is the most feared complication of acute leukemia

Back 27

CNS hemorrhage in a pt with M3 (this is what kills pts early on)

Front 28

dx of AML

Back 28

first, bone marrow exam + biopsy

then, special stain (Wright-Giemsa, Sudan) & flow cytometry/cytogenetics

Front 29

what is the unique tx of M3 AML (acute promyelocytic leukemia)

Back 29

ATRA (all trans retinoic acid) + chemotherapy

Front 30

tx of AML.

name some complications of tx

Back 30

chemotherapy (complications include prolonged neutropenia/thrombocytopenia)

Front 31

when is transplant useful for an AML pt?

Back 31

early on after remission (don't wait for relapse)..bc youll have better outcome.