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acute leukemia Flashcards

1
Q

There are 2 types of acute leukemia:

A
  1. Acute Myelocytic/Myelogenous Leukemia (AML)

2. Acute Lymphocytic/Lymphoblastic Leukemia (ALL)

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2
Q

AML epidemiology

  1. incidence
  2. prevalent in ___ populations
  3. median age
A
  1. greater in industrial countries: 3/100,000
  2. increases with age: 80% oc CML in adults but only 15-20% in children
  3. median age is 68 yrs
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3
Q

ALL epidemiology

  1. incidence
  2. prevalent in ___ populations
  3. median age
  4. common childhood cancer?
A
  1. greater in industrial countries: 1/60,000
  2. incidence declines with age: 75% of ALL in children less than 20% in adults
  3. median age 3-5 years
  4. most common childhood cancer
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4
Q

etiology of leukemia (5)

A
  1. underlying genetic disorders
  2. underlying hematologic disorders
  3. ionizing radiation
  4. chemicals
  5. chemotherapy
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5
Q

clinical presentation of leukemia (5)

A
  1. Bone marrow failure
  2. leukostasis
  3. coagulopathy
  4. extra-medullary menifestations
  5. metabolic abnormalities s
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6
Q

symptoms of?

Non-specific symptoms
Fatigue, malaise, weakness (most common)
Anorexia and weight loss
Fever with or without infection
Bruising/bleeding
Bone pain
A

bone marrow failure

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7
Q

leukostasis:

  1. what is it?
  2. symptoms
  3. more common in? AML vs ALL
A
  1. stasis of blood flow in cerebral and pulmonary circulation especially if the blast count is over 50,000
  2. cerebral sympotms include headache,visual change, confusion, stroke and coma and pulmonary symptoms include SOB, tachypnea and hypoxia
  3. much more common in AML than ALL
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8
Q

coagulopathy

  1. can contribute to bleeding
  2. more common in?
  3. can lead to life threatening
  4. firbinogen, PT and PTT test
A
  1. can contribute to bleeding- DIC
  2. more common in AML than ALL especially with APL subtype
  3. can lead to life threatening intracranial or GI hemorrhage
  4. firbinogen is low PT and PTT are elevated
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9
Q

extra-medullary manifestation in ALL (4)

A
  1. lymphadenopathy and splenomegaly
  2. mediastinal mass
  3. leukemic meningitis rare at diagnosis but CNS is a common site of relapse
  4. testes
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10
Q

Extra-medullary manifesstations in AML (3)

A
  1. leukemia cutis
  2. gingival hypertrophy
  3. chloromas: tumors of blasts
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11
Q

due to rapid cell turn over or lysis from chemotherapy resulting in hyperuricacidemia, hyperphosphatemia, hyperkalemia. Uric acid nephropathy can lead to renal failure.

A

Tumor lysis syndrome (ALL):

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12
Q

due to renal tubular damage by the lysozyme released from myeloblasts

A

hypokalemia in AML

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13
Q

artificial in vitro finding due to metabolic activity of blasts in the blood tube after phlebotomy draw

A

hypoglycemia- metabolic anbnormality

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14
Q
  1. French-American-British (FAB): >___blasts in the bone marrow
    1. World Health Organization (WHO): >____% blasts in the bone marrow
A
  1. French-American-British (FAB): >30% blasts in the bone marrow
    1. World Health Organization (WHO): >20% blasts in the bone marrow
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15
Q

are large immature cells with open chromatin

A

Blasts

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16
Q

have more abundant cytoplasm and may contain granules

A

Myeloblasts

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17
Q

which are linear aggregates of primary granules are seen only in myeloblasts

A

Auer rods- (AML)

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18
Q

Myeloblasts have single to multiple nucleoli where lymphoblasts have

A

Myeloblasts have single to multiple nucleoli where lymphoblasts have less conspicuous nucleoli

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19
Q

AML classification of M3

A

promyelocytic hypergranular

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20
Q

AML classification of M3v

A

microgranular variant- APL

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21
Q
  • Bilobed nucleus
  • Abnormal promyelocytes
  • Large granules
  • “bundles” of Auer Rods
  • t(15;17)
  • DIC
A

Promyelocytic leukemia

AML:M3

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22
Q

small blasts with scanty cytoplasm, inconspicuous nucleoli

A

ALL-L1

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23
Q

larger blasts with abundant cytoplasm, more prominent nucleoli

A

ALL-L2

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24
Q

largest blasts with deep basophilic cytoplasm, prominent nucleoli, often vacuolated

A

ALL-L3

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25
Q
  • Small, homogenous cells
  • Round nuclei
  • Fine chromatin
  • No nucleoli
  • Scant cytoplasm
26
Q
  • Large, heterogeneous cells
  • Clefted, folded nuclei
  • Fine to coarse chromatin
  • 1 or more nucleoli
  • Moderate cytoplasm
27
Q

Large, homogenous cells

  • Oval to round nuclei
  • Dense chromatin
  • 1 or more nucleoli
  • Moderately abundant cytoplasm
  • Prominent vacuoles
  • Burkitt lymphoma
A

ALL-L3:

not really know as a leukemia it is considered more to be burkitt lymphoma

28
Q
  • Lysozyme indicates myeloid differentiation
    • Myeloperoxidase is a cytoplasmic enzyme in the primary granules of myeloblasts
    • Non-specific esterase positive in the monocytic subtype
29
Q

-Terminal deoxynucleotidyl transferase (TdT) is a nuclear enzyme in lymphoblasts

30
Q

Flow cytometry:

CD*13, CD 33, CD 117 on the cell surface

31
Q

flow cytometry:

Mixed phenotype: lineage specific myeloid markers and B or T cell lymphoid markers (5% of acute leukemia)

A

ALL

Procersur B cell (CD19 and 20 and T cell markers (CD 2/3/4/5/7/8)

32
Q

70% of ALL is ___ cell type

33
Q

AML with t(15;17)(q22;q12),

A

(PML/RARalpha)

34
Q

initial management of leukemia: transfusion support

  1. red cells depend on _________
  2. platelets: 10,000 count threshold for prophylactic transfusion to _________
  3. leukocyte depleted to prevent transmission of_____and decreases __________ risk
  4. to prevent ___________ irradiated products should be given
A

this is a required for an effective treatment:

  1. red cells depend on pt.’s clinical status
  2. platelets: 10,000 count threshold for prophylactic transfusion to prevent bleeding
  3. leukocyte depleted to prevent transmission of CMV and decreases alloimmunization risk
  4. to prevent graft vs. host dx. irradiated products should be given
35
Q

Lekostasis is a ________ thus we need to

  1. give
  2. leukapheresis to reduce _________
  3. give hydroxyurea to reduce __________
  4. if pt. has ALL give ______
  5. avoid __________ as it can increase the blood viscosity
A

it is a medical emergency:

  1. give IV fluids
  2. leukapheresis to reduce blast count
  3. give hydroxyurea to reduce leukemic burden
  4. if pt. has ALL give steroids
  5. avoid RBC transfusion as it can increase the blood viscosity
36
Q

Disseminated intravascular coagulopathy managment:

  1. Replacement of clotting factors with __________
  2. Replacement of _________ with cryoprecipitate
  3. _______ transfusion
A
  1. Replacement of clotting factors with fresh frozen plasma
  2. Replacement of fibrinogen with cryoprecipitate
  3. Platelet transfusion
37
Q

Tumor lysis syndrome managment:

  1. ________ for rapid saline diuresis
  2. ________ to help prevent the accumulation of uric acid (blocks conversion of hypoxanthine and xanthine to uric acid)
  3. _________ catalyzes oxidation of uric acid to soluble allantoin
  4. _________ if renal failure develops
A
  1. Intravenous hydration for rapid saline diuresis
  2. Allopurinol to help prevent the accumulation of uric acid (blocks conversion of hypoxanthine and xanthine to uric acid)
  3. Rasburicase catalyzes oxidation of uric acid to soluble allantoin
  4. Hemodialysis if renal failure develops
38
Q

infection management:

  1. Patients often present with fever that could be due to ________
  2. Common infections include _____
  3. All patients with leukemia are at risk
  4. Prophylaxis with (3) is routinely used
A
  1. Patients often present with fever that could be due to underlying infection
  2. Common infections include blood stream infection, sinusitis, bronchitis, pneumonia, urinary track infection
  3. yep
  4. Prophylaxis with antibacterial, anti-fungal and anti-viral medication is routinely used
39
Q

AML-prognostic factors

  1. age
  2. cytogenetics
  3. antecedent bone marrow disorders
  4. treatment
  5. chemo related AML
A
  1. older age is a negative prognostic value; in patients older than 60 there is a survival rate of 10% while those younger than 40 have a 40%
  2. abnormal in 50-80% and it can be divided into three risks
  3. history of bone marrow disorder such as myelodysplastic syndrome, myeloproliferative disorder or aplastic anemia is associated to a poor prognosis (secondary- leukemia)
  4. two phases (1) induction therapy (2) post-remission “consolidation”
  5. alkylating agents and topo II inhibitors
40
Q

t(15;17) in the FAB M3 subtype, acute promyelocyte leukemia (APL):

  1. Fusion of the _____gene on chromosome 15 with the ________ receptor gene on chromosome 17
  2. _______ is a critical ligand in the differentiation pathways
  3. The fusion protein encodes for an aberrant receptor with altered DNA binding and transcription properties leading to a block in __________
  4. Complete remission rate
A
  1. Fusion of the PML gene on chromosome 15 with the retinoic acid receptor gene on chromosome 17
  2. Retinoic acid is a critical ligand in the differentiation pathways mediated through the retinoic acid receptor
  3. The fusion protein encodes for an aberrant receptor with altered DNA binding and transcription properties leading to a block in differentiation
  4. Complete remission rate>90% with high cure rate
41
Q

regulates the expression of genes involved in hematopoietic cell differentiation

A

CBF- core binding factors

42
Q

AML- favorable risk cytogenetics:

  1. t(8;21) results from fusion of the AML1 gene on chromosome 21 (CBFa subunit) with ETO gene on chromosome 8. Common in the _____________ subtype
  2. _______ results in the fusion of the genes for CBFb subunit and smooth muscle heavy chain. Common in the FAB M4 subtype
  3. 2 translocations (5-10% of AML) that affect genes encoding for subunits of the transcription factor called ___________
  4. Complete remission of __________
A
  1. t(8;21) results from fusion of the AML1 gene on chromosome 21 (CBFa subunit) with ETO gene on chromosome 8. Common in the FAB M2 subtype
  2. inv 16 results in the fusion of the genes for CBFb subunit and smooth muscle heavy chain. Common in the FAB M4 subtype
  3. 2 translocations (5-10% of AML) that affect genes encoding for subunits of the transcription factor called core binding factor
  4. Complete remission of 90%, cure 50-60%
43
Q

AML- unfavorable risk cytogenetics

  1. _____ (MLL gene) regulates expression of downstream genes involved in hematopoietic development. Associated with prior treatment with ___________
  2. Deletions of 5 and/or 7. Associated with __________
  3. _________ abnormalities
A
  1. 11q23 (MLL gene) regulates expression of downstream genes involved in hematopoietic development. Associated with prior treatment with topoisomerase inhibitors.
  2. Deletions of 5 and/or 7. Associated with alkylating agents.
  3. Multiple cytogenetic abnormalities
44
Q

AML- intermediate risk cytogenetics:

  1. _______l karyotype
  2. Data now shows that this is a ________ group
  3. When gene mutation analysis such as next generation sequencing, many abnormalities are found: favorable or unfavorable?
    (a) FLT-3 ?
    (b) NPM-1?
    (c) CEBPA ?
A
  1. Normal karyotype
  2. Data now shows that this is a heterogeneous group
  3. When gene mutation analysis such as next generation sequencing, many abnormalities are found:
    (a) FLT-3 (unfavorable risk)
    (b) NPM-1 (favorable risk )
    (c) CEBPA (favorable risk)
45
Q

: initial goal is to quickly induce remission and restore normal hematopoiesis

A

Induction therapy

46
Q

to prevent relapse by eradicating residual leukemia cells

A

Post Remission (Consolidation) therapy:

47
Q

AML induction therapy:

  1. Combination chemotherapy is given which will result in _________
  2. Most common regimen: _____ and an __________(Daunorubicin or Idarubicin). Commonly call “7 and 3”.

3 ________ remission= <5% blasts in the bone marrow with recovery of normal hematopoiesis and resolution of all extra-medullary infiltrates

4 Complete remission in 50-85% of patients but 10-25% die from__________

  1. Hypomethylating agents (decitabine, 5-azacitidine) in ________
A
  1. Combination chemotherapy is given which will result in pancytopenia
  2. Most common regimen: Cytarabine and an anthracycline (Daunorubicin or Idarubicin). Commonly call “7 and 3”.

3 Complete remission= <5% blasts in the bone marrow with recovery of normal hematopoiesis and resolution of all extra-medullary infiltrates

4 Complete remission in 50-85% of patients but 10-25% die from toxicities of treatment

  1. Hypomethylating agents (decitabine, 5-azacitidine) in older patients
48
Q

AML- post remission therapy

  1. Goal of consolidation therapy is
  2. At diagnosis, there are 10^12 _______
  3. Induction lowers the _________by a log
  4. Usually 1-4 cycles of chemotherapy are given that are similar but less.more intense than induction therapy
A
  1. Goal of consolidation therapy is to prevent relapse and prolong survival by eradicating residual leukemia cells
  2. At diagnosis, there are 1012 leukemic blasts
  3. Induction lowers the disease burden by a log
  4. Usually 1-4 cycles of chemotherapy are given that are similar but less intense than induction therapy
49
Q

AML- survival

  1. Overall survival for AML at 5 year is about _______
  2. For favorable risk patients, 50-60% are likely cured with __________
  3. For intermediate and poor risk patients, ______________ is performed frequently
    • Cure rate is variable depending on age, remission status
  4. Survival after relapse remains_________
A
  1. Overall survival for AML at 5 year is about 25%
  2. For favorable risk patients, 50-60% are likely cured with chemotherapy
  3. For intermediate and poor risk patients, hematopoietic stem cell transplantation is performed frequently
    • Cure rate is variable depending on age, remission status
  4. Survival after relapse remains poor
50
Q

Acute Promyelocytic Leukemia

  1. APL is ____% of AML cases
  2. Important to recognize because the _________ is different
  3. Patients are ________, often ages 30-40
  4. Usually present with ____________
  5. Often have __________ at diagnosis
  6. Blasts have numerous primary granules and ____________

7.Presence of t(15;17) or detection of the PML/RAR fusion product by PCR confirms the diagnosis
ATRA (all-trans retinoic acid), Vitamin A analog

  1. Ligand for the RAR receptor, binds to ____________ protein
  2. Restores transcription of genes that allow ________
  3. Does not cause lysis of the blasts or marrow aplasia
  4. APL Differentiation syndrome: Leukocytosis, fever, pulmonary infiltrates. Can be fatal if not recognized and treated with_________
  5. The addition of an ___________decreases APL Differentiation syndrome from 25% to 5%
A
  1. APL is 5-15% of AML cases
  2. Important to recognize because the treatment is different
  3. Patients are younger, often ages 30-40
  4. Usually present with leukopenia
  5. Often have DIC at diagnosis
  6. Blasts have numerous primary granules and Auer rods

7.Presence of t(15;17) or detection of the PML/RAR fusion product by PCR confirms the diagnosis
ATRA (all-trans retinoic acid), Vitamin A analog

  1. Ligand for the RAR receptor, binds to PML-RAR fusion protein
  2. Restores transcription of genes that allow differentiation
  3. true
  4. APL Differentiation syndrome: Leukocytosis, fever, pulmonary infiltrates. Can be fatal if not recognized and treated with steroids.
  5. The addition of an anthracycline decreases APL Differentiation syndrome from 25% to 5%
51
Q

Acute promyelocytic leukemia:

  1. __________(ATO) also effective
  2. Induces apoptosis in the_____ cells and targets the PML/RAR fusion protein inducing ___________
  3. Cardiac toxicities:
  4. APL Differentiation syndrome
  5. Standard treatment: __________and ____________ for induction. Complete remission >90%.
  6. Maintenance therapy with ATRA and oral anti-metabolite: long term remission in ___%.
  7. ___________now also considered standard of care: 99% 2 year survival
A
  1. Arsenic Trioxide (ATO) also effective
  2. Induces apoptosis in the APL cells and targets the PML/RAR fusion protein inducing differentiation
  3. Cardiac toxicities: prolongation of the QTc interval, toursades de points, Atrio-ventricular block
  4. APL Differentiation syndrome
  5. Standard treatment: ATRA and anthracycline for induction. Complete remission >90%.
  6. Maintenance therapy with ATRA and oral anti-metabolite: long term remission in 70%.
  7. ATO with ATRA now also considered standard of care: 99% 2 year survival
52
Q

ALL prognostic factors:

  1. age
  2. immunophenotype better prognosis
  3. other prognostic values
  4. treatment (3)
A
  1. excellent prognosis in children 1-0 but worse for infants and adults. overall survival rate is 10%
  2. B cell ALL has better prognosis than T
  3. High WBC (30,000 in B cell, 100,000 in T cell)
    Time to remission
    Minimal residual disease at end of induction therapy
    Sex: girls better than boys
  4. treatment: induction. intensification, maintenance
53
Q

ALL cytogenetics favorable or unfavorable:

t(12;21): fusion of Tel and AML1. Found in 25% of children but rare in adults.
Hyperploidy (more than 50 chromosomes)
Normal cytogenetics

54
Q

ALL cytogenetics favorable or unfavorable:

t(9;22): the Philadelphia chromosome. Found in 20% adults but rare in children.
Translocations involving the MLL gene on 11. Found in 5% of patients usually infants and adults.
Hypoploidy (less then 44 chromosomes)

A

unfavorable

55
Q

: Induce remission and restore normal hematopoiesis

A

induction- ALL

56
Q

): reduce total body leukemia burden

A

Intensification (consolidation

57
Q

eradicate minimal residual disease and prolong survival»CURE!

A

ALL maitenance

58
Q

ALL induction therapy:

  1. Multi-agent chemotherapy is given to induce a____
  2. associated with
  3. ___________inhibitors in Ph+ (imatinib, dasatinib)
  4. Complete Remission:
A
  1. Multi-agent chemotherapy is given to induce a complete remission
  2. Vincristine, glucocorticoid, anthracycline, L-Asparaginase, Cytarabine, Cyclophosphamide, Methotrexate
  3. Tyrosine kinase inhibitors in Ph+ (imatinib, dasatinib)
  4. Complete Remission: 65-95%
59
Q

all intensification :

  1. ___________ given after achieving a complete remission

2___________is a sanctuary site for leukemic blasts

  1. CNS prophylaxis with intrathecal chemotherapy with or without craniospinal irradiation is __________
A
  1. Chemotherapy given after achieving a complete remission
  2. Cerebrospinal fluid is a sanctuary site for leukemic blasts
  3. CNS prophylaxis with intrathecal chemotherapy with or without craniospinal irradiation is necessary
60
Q

ALL maintenance therapy

  1. Prolonged _____________ has been shown to extend survival and improve cure rates.
  2. Consists of daily oral plus weekly ——
  3. Total duration of therapy is usually _______ (longer in boys)
A
  1. Prolonged chemotherapy has been shown to extend survival and improve cure rates.
  2. Consists of daily oral plus weekly chemotherapy
  3. Total duration of therapy is usually 2-3 years (longer in boys)
61
Q

ALL survival

A

most children cured while adults do wrose

heme/onc (30 decks)

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