14 - Myelodysplastic Syndromes and Acute Leukemias Flashcards

1
Q

myelodysplastic syndrome definition

A

spectrum of clonal myeloid disorders characterized by:
ineffective hematopoiesis
cytopenias
qualitative disorders of blood cells
clonal chromosomal abnormalities
variable tendency to evolve into acute leukemia

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2
Q

most common clonal hematologic disorder

A

myelodysplastic syndrome

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3
Q

age/gender more likely to get MDS

A

men, old (increases logarithmically with age)

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4
Q

typical type of mutation causing MDS

A

chromosomal deletions or additions, often chromosomes 5 or 7

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5
Q

risk factors for MDS

A

radiation
drugs/toxins - benzene, alkylating agents, topoisomerase 2 inhibitorss, smoking, hair dye, pesticides etc
other hematologic disorders - aplastic anemia, PNH, congential neutropenia
genetic disorders - Downs, fanconi, NF

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6
Q

clinical manifestations of MDS

A

anemia sx, thrombocytopenia (bleeding/bruising), leukopenia (infxns), polyarthritis, neuropathy, splenomegaly, acquired alpha thalassemia

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7
Q

PBS findings in MDS

A

RBCs - anisocytosis, normo-mildly macrocytic, low retics, Howell Jolly bodies, Cabot rings, basophilic stipling
WBCs - small poorly granulated neutrophils w/ hyper or hypo lobed nuclei
platelets - large and agranular

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8
Q

isolated del(5q)

A
MDS
primarily in middle aged women
macrocytic anemia and normal/inc platelets
megakaryocytes have hypolobulated nuclei
favorable clinical course
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9
Q

supportive care for MDS

A

EPO, G-CSF, GM-CSF, IL-11, transfusions, iron chelation, pyridoxine, folic acid, danazol
immunomodulation - cyclosporine, steroids
AML style chemo

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10
Q

5-azacytidine (vidaza) and decitabine (dacogen)

A

vidaza is prodrug version of dacogen
reduces DNA methylation > allows reactivation of silenced tumor suppressors
significant side effects - cytopenias, GI, fever, fatigue, aches/pains

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11
Q

general characteristics of acute leukemias

A

clonal - originate from transformed hematopoietic stem cell
malignant cells do not differentiate or do so abnormally > accumulate in blood, marrow, tissues
inhibits normal hematopoiesis
fatal if untreated

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12
Q

who gets more AML - adults or children

A

adults

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13
Q

risk factors for AML

A

genetic - Downs, klinefelter, fanconi, Bloom’s
prior chemo - alkylating agents and topoisomerase II inhibs
radiation
benzene
myelodysplasia, MPDs

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14
Q

important translocations assoc w/ AML

A

t(8,21) - AML w/ maturation (M2)
t(15,17) - acute promyelocytic leukemia (M3)
inv(16) - acute myelomonocytic leukemia (M4)

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15
Q

t(8,21) is assoc w/

A

AML w/ maturation (M2)

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16
Q

t(15,17) is assoc w/

A

acute promyelocytic leukemia (M3)

17
Q

inv(16) is assoc w/

A

acute myelomonocytic leukemia (M4)

18
Q

clinical presentation of AML

A

cytopenias
hyperleukocytosis (inc risk of CNS involvement)
extramedullary dz (gums in M4/5, chloromas w/ M2, CNS)
coagulopathy

19
Q

gum infiltration can be seen with

A

AML (M5)

20
Q

prognosis (general good/bad/ugly) for AMLs M2, M3, M4

A

all good

21
Q

M3 AML

A

acute promyelocytic, t(15,17)
seen in younger hispanic pts
risk of DIC relatively high
tx w/ all trans retinoic acid

22
Q

tx of AML

A

induction chemo - anthracycline + cytarabine
consolidation chemo
bone marrow transplant

23
Q

special tx for M3 AML

A

ATRA (all trans retinoic acid) + (chemo OR arsenic)

24
Q

prognosis for AML - remission vs long term cure for adults and children

A

adults - 70% remission, 30% cure

children - 90% remission, 40% cure

25
Q

ALL is more common in kids or adults?

A

kids

26
Q

risk factors for ALL

A

in utero events, radiation, SES (higher > higher risk), ethnicity (hispanic)

27
Q

ALL is more often B or T cells?

A

B

28
Q

presentation of ALL

A

fatigue, malaise, arthralgia, bone pain, lymphadenopathy, hepatosplenomegaly, fever/infxn, bleeding, CN/neuro abnormalities

29
Q

good prognostic factors for ALL

A

female, age 3-8, low WBC, Pre-B phenotype, C10, low LDH, hyperdiploid cytogeneitcs

30
Q

tx of ALL

A

one drug is NEVER enough (most drugs are active against it)
tx for at least 18 mo
get sanctuary sites (CNS)
more risk > more intense tx
early response is very important prognostic factor
relapse is bad sign

induction chemo, consolidation chemo, re-induction, maintenance w/ POMP, BM transplant

31
Q

Burkitt lymphoma assoc genetic mutations

A

in the c-myc gene -
t(2;8)
t(8;14)
t(8;22)

32
Q

mutations in c-myc gene assoc w/

A

burkitt lymphoma

33
Q

tumor lysis syndrome and prevention

A

renal failure due to urate nephropathy and CaPO4 crystals

can be prevented w/ hydration, allopurinol, alkalinization, recombinant uricase

34
Q

are CNS findings more common in ALL or AML

A

ALL