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37 Cards in this Set

  • Front
  • Back
What's the prognosis for AML?
3/4 of people will die from it.
What causes AML?
Environmental factors: chemicals (benzene,carbon tet), radiation
Chemo drugs: topoII inhibitors, alkylating agents
Genetic abnormalities: Down's, Fanconi's
Spontaneous bone marrow failure disorders: AA, paroxysmal nocturnal hemoglobinuria
Malignant bone marrow disorders
What are the symptoms of acute myeloid leukemia/
Too many blasts
Anemia (and symptoms)
Neutropenia (and symptoms)
Thrombocytopenia (and symptoms)

Leukostasis
Extramedullary disease
DIC
Electrolyte abnormalities (hyper everything but calcium)
What are the symptoms of leukostasis?
Headaches
Vision changes
Chest pain
SOB
Neuropathy
Abdominal angina
Priapism
What's the definition of acute myeloid leukemia?
>20% myeloblasts in either the periphery or the marrow
What's the definition of acute lymphoblastic leukemia?
>20% lymphoblasts in the periphery or marrow
How do you define a myeloid phenotype?
Auer rods
Myeloperoxidase
Monocytic differentiation proteins
How do you establish a monocytic differentiation?
CD11c
CD14
CD64
Lysozyme

Don't need to memorize these!
What's the difference between FAB and WHO classification?
FAB: just on morphology

WHO: genetic changes to give prognosis information
What should you do if your patient has AML?
Hospitalize them!

It's a medical emergency.
What re the different phases of AML treatment?
1. Induction: getting them into remission
2. Consolidation: keeping them in remission
What drugs are given during the induction phase of AML treatment?
Anthracycline for 3 days
Cytarabine for 7 days

3+7 regimen

You're taking the blood counts to zero.
What is the definition of remission from AML?
1. Return of the counts back to normal
2. No more leukemia cells.
What are the complications to AML induction therapy?
DIC
Tumor lysis syndrome
Nausea, vomiting
Myelosuppression requiring transfusion support
Mucositis (no mucosal regeneration)
Neutropenic infections
Cardiac toxicity

Death in 5%
What do you have to do before you can do induction therapy for AML?
Determine cardiac status

The drugs are cardiotoxic, so you need to make sure that they can handle it.
What's the outcome from induction therapy in AML?
60-70% of people achieve remission
What happens after induction therapy for AML?
Almost all of them die, anyways.
What do you do in consolidative therapy for AML?
1. Additional chemo
2. alloHSCT
What gives someone the best prognosis with AML? Worst?
Best: t(8,21), i(16); the core binding factor leukemias
Intermediate: normal karyotype
Worst: complex karyotype

The more mutations to the chromosomes, the worse the prognosis
What's the prognosis for people in the core binding facotor mutations in AML?
40% survival

Still not a success.
What are some genes that we look at in AML that determine prognosis? In what scenarios are they impactful?
Nucleophosmin: NPM1
FMS-like TRK
CEBPalpha

Good prognosis
Normal karyotype: NPM1 with normal FLT3 (75% survival)
CEBPalpha

Bad prognosis;
NPM1 with a FCT3 mutation (25% survival)
What's the difference in treatment of AML with a good prognosis vs. bad?
Good prognosis: chemotherapy

Bad prognosis: bone marrow transplant
Who should get transplant with AML?
Poor risk karyotypes (abnormal karyotype)/molecular diagnostics

Primary refractory disease
Secondary AML
Therapy-AML
What type of leukemia has its own induction therapy?
Acute promyelocytic leukemia
What is the karyotypic mutation in acute promyelocytic leukemia?
t(15;17)

It's necessary and sufficient for diagnosis
What is the age of diagnosis in acute promyelocytic leukemia?
30-40
What ethnicity gets acute promyelocytic eukemia?
Spanish people
What's a common presentationfor acute promyelocytic leukemia?
Leukopenia

DIC
What should you do if you see someone who has acute promyelocytic leukemia?
HOSPITALIZE THEM!
What should make you suspect acute promyelocytic leukemia on a smear?
Multiple auer rods
Sliding plate nuclei

Or, promyelocytes
Multiple auer rods
Sliding plate nuclei

Or, promyelocytes
What's necessary to diagnose acute promyelocytic leukemia?
Identification of t(15;17) via

Karyotyping
FISH
RT-PCR for gene product
What's the fusion gene product in acute promyelocytic leukemia?
A binding protein to the retinoic acid binding region
What's the treatment for acute promyelocytic leukemia? How does it work?
All trans retinoic acid
Arsenic trioxde

They bind to the protein and don't let it bind to the promoter.
What's the secondary problems with treatment of APL?
Rapid differentiation of the cells causes a capillary leak syndrome, in which the plasma doesn't stay in the blood cells

YOU NEED TO SEE THIS.

Discontinue ATRA if you see the capillary leak syndrome
What are the symptoms of capillary leak syndrome?
Weight gain
Fever
Hypoxia
Pleuro/pericardial effusions
Pulmonary infiltrates
What are the ways that you can approach a patient with APL?
ATRA + chemo

ATRA+arsenic

Just arsenic

The outcomes aren't yet determined for each vs. each other.
What's the big problem with 3+ 7 therapy?
Lots of mortality in people who are old:

20% mortality above 60
40% mortality above 80.