Aml

Front 1

keywords for AML?

Back 1

malignant disease of the bone marrow in which hematopoietic precursors are arrested in an early stage of development

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incidence of AML?

Back 2

2.5/100'000, rising with increasing age

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what proportion of AML affects adults?

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80%

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median age in AML?

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60 years

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principal pathophysiology in AML?

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maturational arrest of bone marrow cells in earliest stages of development <-- in many cases activation of abnormal genes through chromosomal translocations and other genetic abnormalities

Front 6

major disease processes in AML?

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marked decrease in production of normal blood cells --> anemia, thrombocytopenia, neutropenia

rapid proliferation of arrested cells with accumulation in the bone marrow, blood and frequently liver and spleen

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M:F in AML?

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M>F, likely because MDS more frequent in men and advanced MDS often with progression to AML

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principal groups of clinical symptoms in AML?

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symptoms from bone marrow failure

and/or

symptoms from organic infiltration with leukemic cells

Front 9

major symptoms of bone marrow failure in AML?

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anemia, neutropenia, thrombocytopenia -->

fatigue, history of persistent upper respiratory infections and bleeding

Front 10

most common sites of organic infiltration with leukemic cells in AML?

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spleen, liver, gums, skin

Front 11

organic infiltration most common in which forms of AML?

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monocytic subtypes

Front 12

symptoms from splenomegaly?

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fullness in LUQ and early satiety

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symptoms from gum infiltration?

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gingiva hyperplasia, gingivitis, gum bleeding

Front 14

symptoms from leukostasis (hyperviscosity syndrome)?

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triad of bleeding diathesis, visual disturbances and focal neurologic signs

bleeding: epistaxis or gingivorrhagia

visual disturbances: decreased visual acuity, distended or thrombotic veins, papillary edema

focal neurologic signs: dizziness, seizures, coma, stroke

additionally respiratory distress altered mental status and priapism

Front 15

which of the hematologic disorders is the most ccommon risk factor for AML?

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presence of antecedent hematologic disorder, most common of which is MDS

others are aplastic anemia, MPN (myelofibrosis, PV) and paroxysmal nocturnal hemoglobinuria

Front 16

congenital disorders presenting a risk factor for AML?

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Down syndrome
Bloom syndrome
Fanconi anemia
neurofibromatosis

--> usually AML during childhood

Front 17

environmental exposures presenting a risk factor for AML?

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radiation exposure

smoking (OR 1.5)

benzene (=Benzol) exposure

Front 18

major groups of risk factors for AML?

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previous exposure to chemotherapeutic agents
antecedent hematologic disorder
congenital disorders
environmental exposures


mnemonic: PACE

Front 19

types of chemotherapy as risk factor for AML?

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alkylating agents
often MDS then AML

topoisomerase-II inhibitors
no MDS before AML

Front 20

criteria for diagnosis of acute leukemia?

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>20% blasts in the bone marrow

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most common subtype of AML associated with DIC?

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acute promyelocytic leukemia (APL) = M3

Front 22

what are the major WHO classification groups in AML?

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AML with recurrent genetic abnormalities

AML with myelodysplasia-related changes

therapy-related myeloid neoplasms

AML, NOS (not otherwise specified)

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object of induction therapy?

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complete hematologic remission

i.e. blasts <5%, "normalisation " of peripheral blood count

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common induction therapy in AML exc M3?

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combination therapy

ara-C plus anthracycline (eg daunorubicin, idarubicin)

mnemonic: AML --> American Airlines = AA

Front 25

object of consolidation therapy in AML?

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prevention of relapse

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options in consolidation therapy in younger patients in AML?

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chemotherapy, eg high-dose ara-C

autologous stem-cell transplantation

allogeneic stem cell transplantation

Front 27

what are the classification systems for AML?

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WHO 2008

FAB

Front 28

what is the basis of the WHO classification of AML?

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cytogenetic, molecular and immunphenotype information

Front 29

what is the basis of the FAB classification of AML?

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morphology and histochemistry

Front 30

what is the classification of AML according to FAB?

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M0 acute undifferentiated leukemia
M1 acute myeloblastic leukemia
M2 acute myeloblastic leukemia with differentiation
M3 acute promyelocytic leukemia (APL)
M4 acute myelomonocytic leukemia
M5 acute monoblastic leukemia
M6 erythroleukemia
M7 megakaryoblastic leukemia

Front 31

what is AML M3 and what are characteristic findings?

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acute promyelocytic leukemia (APL)

cytogenetic findings are t(15;17) and the fusion gene PML-RAR alpha

Front 32

what are the most powerful prognostic factors in AML?

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cytogenetic studies

Front 33

of what risk are the majority of AML?

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intermediate risk with either normal cytogenetics or abnormalities that do no confer strong prognostic significance

Front 34

what are cytogenetic finding confering poor prognosis in AML?

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monosomy 5 or 7 or complex cytogenetics with more that threee separate abnormalities

Front 35

what is AML M3 and what are characteristic findings?

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acute promyelocytic leukemia (APL)

cytogenetic findings are t(15;17)(q22;q12) with the fusion gene PML-RAR alpha

mnemonic: Promyelocytic-(A)ML, not so RARe

Front 36

what are the most powerful prognostic factors in AML?

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cytogenetic studies

Front 37

of what risk are the majority of AML?

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intermediate risk with either normal cytogenetics or abnormalities that do no confer strong prognostic significance

Front 38

what are cytogenetic finding confering poor prognosis in AML?

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monosomy 5 or 7 or complex cytogenetics with more than three separate abnormalities

Front 39

what is the treatment for the AML variant APL?

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induction therapy with an anthracycline plus all-trans-retinoic acid (ATRA)

Front 40

what is the median age in APL?

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40 years, thus considerably lower median age than other subtypes

Front 41

with what finding is bleeding in APL frequently associated

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DIC

Front 42

what is the prognosis in AML for adults <60 years?

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mnemonic: 80-half-half

80% under age 60 with complete remission

with high-dose remission therapy half of those with cure (40%)

allogeneic bone marrow transplantation curative in half of cases

Front 43

what is the prognosis in AML for adults >60 years?

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50% over age 60 with complete remission

cure rate in only 10%

mnemonic: 50 + 10 = 60

Front 44

what are the principle therapy steps particular in APL?

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induction therapy, followed by a consolidation therapy, also called postremission therapy and aditionally a maintenance therapy

Front 45

what does the maintenance therapy in APL consist of?

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6-Mercaptopurin daily

Methotrexat weekly

ARTRA intervals every 3 months

mnemonic: maintenance therapy with MiXed Martial ARTs = MMA

Front 46

what is the pathognomonic finding in the blood smear of AML?

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Auer rods, which is an eosinophilic needle-like inclusion in the cytoplasm of blasts

Front 47

what are the phenotypic findings in AML?

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myeloid antigens such as CD13 or CD33

Front 48

what is the method to determine the immunophenotype of the leukemia?

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flow cytometry

Front 49

what are the histochemic findings in AML?

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peroxidase in myeloid cells and butyrate esterase in monocytic cells

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what is the differential diagnosis to AML?

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other myeloproliferative disorders, such as CML and myelodysplastic syndrome