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ALL Flashcards

1
Q

HIGH-RISK FEATURES B CELL ALL

A
  • Age >35 years
  • White blood cell (WBC) count >30 x 109/L
  • Poor Risk Cytogenics and Molecular
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2
Q

HIGH-RISK FEATURES T CELL ALL

A
  • Age >35 years
  • White blood cell (WBC) count >100 x 109/L
  • ETP-ALL
  • RAS/PTEN mutation and/or NOTCH1/FBXW7 wildtype
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3
Q

Standard risk Cytogenetic and Molecular Alterations

A
  • Hyperdiploidy (51–65 chromosomes)
  • t(12;21)(p13;q22): ETV6::RUNX1i
  • t(1;19)(q23;p13.3): TCF3::PBX1
  • DUX4 rearranged
  • PAX5 P80R
  • t(9;22)(q34;q11.2): BCR::ABL1j without IKZF1 plus and without antecedent chronic myeloid leukemia (CML)

Cases with trisomy of chromosomes 4, 10, and 17 appear to have the most favorable outcome

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4
Q

Poor risk Cytogenetic and Molecular Alterations

A
  • Hypodiploidyl,m (<44 chromosomes)
  • TP53 mutation
  • KMT2A rearranged (t[4;11] or others)
  • IgH rearranged
  • HLF rearranged
  • ZNF384 rearranged
  • MEF2D rearranged
  • MYC rearranged
  • BCR::ABL1-like (Philadelphia chromosome [Ph]-like) ALL
  • PAX5alt
  • t(9;22)(q34;q11.2): BCR::ABL1 with IKZF1 plus and/or antecedent CML
  • Intrachromosomal amplification of chromosome 21 (iAMP21)
  • Alterations of IKZF1
  • Complex karyotype (5 or more chromosomal abnormalities)

BCR::ABL1-like (Philadelphia chromosome [Ph]-like) ALL
* JAK-STAT (CRLF2r,o EPORr, JAK1/2/3r, TYK2r, mutations of SH2B3, IL7R, JAK1/2/3)
* ABL class (rearrangements of ABL1, ABL2, PDGFRA, PDGFRB, FGFR)
* Other (NTRKr, FLT3r, LYNr, PTK2Br)

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5
Q

The first LP be performed at

A

Time of initial scheduled IT therapy

Unless directed by symptoms to perform earlier

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6
Q

AYA age range

A

15–39 years

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7
Q

TKI options for Ph+ ALL

A

Bosutinib, dasatinib, imatinib, nilotinib, or ponatinib

Not all TKIs have been directly studied within the context of each specific regimen and the panel notes that there are limited data for bosutinib in Ph+ ALL.

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8
Q

Post HCT TKI

TKI should be continued for at least _____ years post-HCT.

A

2 years

The recommended duration of TKI during maintenance chemotherapy is at least until completion of maintenance chemotherapy.

The optimal duration of TKI is unknown in both settings.

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9
Q

Preferred regmen for Ph- B/ T cell ALL AYA patients

A

Pediatric-inspired regimens

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10
Q

For patients in late relapse (______ years from initial diagnosis), consider treatment with the same induction regimen

A

> 3 years

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11
Q

TRUE OR FALSE

The panel recommends that IT therapy be administered with initial LP.

A

TRUE

The panel recommends that IT therapy be administered with initial LP.

All patients with ALL should receive CNS prophylaxis.

Although the presence of CNS involvement at the time of diagnosis is uncommon (about 3%–7%), a substantial proportion of patients (>50%) will eventually develop CNS leukemia in the absence of CNS-directed therapy.

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12
Q

Classification of CNS status:

A
  • CNS-1: No lymphoblasts in cerebrospinal fluid (CSF) regardless of WBC count
  • CNS-2: WBC < 5/mcL in CSF with presence of lymphoblasts.
  • CNS-3: WBC ≥ 5/mcL in CSF with presence of lymphoblasts.

STEIHERZ-BLEYER ALGORITH If the patient has leukemic cells in the peripheral blood, the LP is traumatic, and WBC ≥5/mcL in CSF with blasts, then compare the CSF WBC/red blood cell (RBC) ratio to the blood WBC/RBC ratio.

If the CSF ratio is at least two-fold greater than the blood ratio, then the classification is CNS-3; if not, then it is CNS-2.

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13
Q

CNS-directed therapies

A
  • Cranial irradiation
  • IT therapy (eg, methotrexate, cytarabine, corticosteroid), and/or
  • Systemic chemotherapy (eg, high-dose methotrexate, intermediate or high-dose cytarabine, pegaspargase [PEG])
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14
Q

Indication for CNS irradiation

A
  • CNS leukemia (CNS-3 and/or cranial nerve involvement) at diagnosis, or persisting after induction
  • Overt CNS leukemia at diagnosis
  • Relapsed/refractory therapy settings

18 Gy in 1.8–2.0 Gy/fraction

The entire brain and posterior half of the globe should be included. The inferior border should include C2.

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15
Q

Testicular radiation dose

A

24 Gy in 2.0 Gy/fraction

Pag may tira after systemic

Patients with clinical evidence of testicular disease at diagnosis that is not fully resolved by the end of the induction therapy should be considered for radiation to the testes in the scrotal sac, which is typically done concurrently with the first cycle of maintenance chemotherapy.

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16
Q

TRUE OR FALSE

Trimethoprim/sulfamethoxazole may be held when high-dose methotrexate is administered and restarted when methotrexate clearance is achieved.

A

TRUE

Trimethoprim/sulfamethoxazole may be held when high-dose methotrexate is administered and restarted when methotrexate clearance is achieved.

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17
Q

Drug given if a patient receiving high-dose methotrexate experiences delayed elimination due to renal impairment

  • Plasma methotrexate concentrations are two standard deviations above the mean expected plasma concentration as determined by MTXPK.org OR
  • plasma methotrexate level is >30 μM at 36 hours, >10 μM at 42 hours, or >5 μM at 48 hours.
A

Glucarpidase

Optimal administration of glucarpidase is within 48 to 60 hours from the start of methotrexate infusion.

Leucovorin should be continued for at least 2 days following glucarpidase administration and should be administered at least 2 hours before or 2 hours after glucarpidase.

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18
Q

Drug given to patients who develop veno-occlusive disease (VOD) related to inotuzumab ozogamicin toxicity.

A

Defibrotide

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19
Q

TRUE OR FALSE

For those patients receiving inotuzumab ozogamicin as a bridge to allogeneic HCT, double alkylator conditioning is strongly discouraged.

A

TRUE

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20
Q

May be considered for VOD prophylaxis in use of Inotuzumab Ozogamicin

A

Ursodiol

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21
Q

Complication of Blinatumomab and Tisagenlecleucel/ Brexucabtagene Autoleucel:

A systemic inflammatory condition characterized by fever or hypothermia, that may progress to hypotension, hypoxia, and/or end organ damage.

A

Cytokine release syndrome (CRS)

Infusion should be held with consideration for steroids and/or vasopressors for those with severe symptoms in accordance with manufacturer guidelines and prescriber information.

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22
Q

Preferred for patients with severe CRS

A

Tocilizumab

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23
Q

Preferred for tocilizumab-refractory CRS and/or neurologic toxicity

A

Steroids

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24
Q

There are three formulations of asparaginase in clinical use:

A
  • 1) PEG
  • 2) calaspargase pegol-mknl (Cal-PEG) (in patients aged ≤21 years)
  • 3) asparaginase Erwinia chrysanthemi (recombinant)-rywn (ERW-rywn)

The preferred route for administration for both PEG and Cal-PEG is IV

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25
Q

The panel recommends that the dose of PEG or Cal-PEG should be capped at ____________.

A

One vial (3750 IU)

26
Q

Management of anaphylaxis or other allergic reactions of Grade 3–4 severity with Asparaginase

A

Permanent discontinuation of the type of asparaginase that caused the reaction.

27
Q

Management of Grade 1 reactions and Grade 2 reactions with Asparaginase (rash, flushing, urticaria, and drug fever ≥38°C) without bronchospasm, hypotension, edema, or need for parenteral intervention

A
  • The asparaginase that caused the reaction may
    be continued,
  • Anti-allergy premedication (such as hydrocortisone, famotidine or ranitidine, diphenhydramine, cetirizine, and
    acetaminophen).
28
Q

Measures that can be considered for preventing or limiting severity of infusion reactions or hypersensitivity reactions

A
  • Slowing the infusion to ≥ 2 hours
  • Infusing normal saline concurrently
  • Use of premedications
29
Q

For patients receiving 6-MP, consider testing for _________________ , particularly in patients who develop severe neutropenia after starting 6-MP.

A

Thiopurine methyltransferase (TPMT) gene polymorphisms

Testing for both TPMT and NUDT15 variant status should be considered, especially for patients of East Asian origin.

30
Q

Preferred Regimens Ph- ALL AYA Patients

A
  • CALGB 10403 regimen: daunorubicin, vincristine, prednisone, and PEG
  • DFCI ALL regimen based on DFCI Protocol 00-01: doxorubicin, vincristine, prednisone, high-dose methotrexate, and PEG
31
Q

Preferred Regimens Ph- ALL Adult Patients (<65 years and without substantial comorbidities)

A
  • ECOG1910: daunorubicin, vincristine, prednisone, and PEG (induction phase I); and cyclophosphamide, cytarabine, and 6-MP (induction phase II) + blinatumomab; with rituximab for CD20-positive disease
32
Q

Maintenance Regimens for Ph-Negative B- ALL

A
  • Weekly methotrexate/daily 6-MP/monthly vincristine/prednisone pulses (duration based on regimen)
  • Blinatumumab alternating with POMP
33
Q

Preferred Regimens T- ALL AYA Patients

A
  • CALGB 10403: daunorubicin, vincristine, prednisone, and PEG
  • COG AALL0434: daunorubicin, vincristine, prednisone, PEG; ± nelarabine
34
Q

Preferred Regimens for Relapsed and Refractory T- ALL

A

Nelarabine ± etoposide and cyclophosphamide

35
Q

Treatment of Adults ≥65 Years/ with Comorbidities: Frontline and R/R

The categorization of regimens as low, moderate, or high intensity is based on two factors:

A
  • 1) the presence or absence of myelosuppressive cytotoxic agents
  • 2) the relative dose intensity of the included agents

Dose reduction of pegylated asparaginase (1000 IU/m2), anthracycline (50% dose), and/or other myelosuppressive agents may be warranted.

36
Q

Treatment of Adults ≥65 Years/ with Comorbidities: Frontline and R/R

Low Intensity

A
  • Vincristine/prednisone
  • POMP
37
Q

Treatment of Adults ≥65 Years/ with Comorbidities: Frontline and R/R

Moderate Intensity

A
  • EWALL regimen
  • GMALL regimen
  • GMALL regimen + rituximab (for CD20-positive disease)
  • PETHEMA ALLOLD07 regimen
  • GRAALL regimen
  • Modified DFCI 91-01 protocol
  • Inotuzumab ozogamicin + mini-hyperCVD
  • Inotuzumab/ozogamicin/mini-hyper-CVD/blinatumomab
38
Q

Treatment of Adults ≥65 Years/ with Comorbidities: Frontline and R/R

High Intensity

A
  • Hyper-CVAD with dose-reduced cytarabine to 1 g/m2
  • Hyper-CVAD with dose-reduced cytarabine to 1 g/m2/blinatumomab
  • CALGB 9111 regimen
  • ECOG 1910 regimen
39
Q

Response Criteria for Blood and Bone Marrow

Complete remission (CR)

A
  • No circulating lymphoblasts or extramedullary disease
  • No lymphadenopathy, splenomegaly, skin/gum infiltration, testicular mass, CNS involvement
  • Trilineage hematopoiesis (TLH) and <5% blasts
  • Absolute neutrophil count (ANC) ≥1000/microL
  • Platelets ≥100,000/microL
40
Q

Response Criteria for Blood and Bone Marrow

CR with partial hematologic recovery (CRh)

A

Meets all criteria for CR except with partial recovery of peripheral blood counts (platelets ≥50,000/microL and ANC ≥500/microL)

41
Q

Response Criteria for Blood and Bone Marrow

CR with incomplete hematologic recovery (CRi)

A

Meets all criteria for CR except without recovery of platelet count or without recovery of ANC (platelets <100,000/microL and ANC ≥1000/microL or platelets ≥100,000/microL and ANC <1000/microL)

42
Q

Response Criteria for Blood and Bone Marrow

Morphologic leukemia-free state (MLFS)

A
  • Blasts <5% and no measurable extramedullary leukemia
  • ANC <500/microL and platelets <50/microL
  • The marrow shows ≥10% cellularity, with at least 200 cells enumerated from an aspirate that contains spicules
43
Q

Response Criteria for Blood and Bone Marrow

Aplastic marrow

A

All criteria for MLFS are met, but with <10% cellularity and/or an aspicular aspirate with <200 cells that can be enumerate

44
Q

Response Criteria for Blood and Bone Marrow

Refractory disease

A

CR not achieved at the end of induction

45
Q

Response Criteria for Blood and Bone Marrow

Progressive disease (PD)

A

Appearance of circulating leukemic blasts or an increase of at least 25% in the absolute number of circulating or bone marrow blasts or development of extramedullary disease

46
Q

Response Criteria for Blood and Bone Marrow

Relapsed disease

A

Reappearance of blasts in the blood or bone marrow (>5%) or in any extramedullary site after a CR

47
Q

Response Criteria for CNS Disease

CNS remission:

CNS relapse:

A

CNS remission: Achievement of CNS-1 status in a patient with CNS-2 or CNS-3 status at diagnosis.

CNS relapse: New development of CNS-2 or CNS-3 status or clinical signs of CNS leukemia such as facial nerve palsy, brain/eye involvement, or hypothalamic syndrome without another explanation.

48
Q

Response Criteria for Lymphomatous Extramedullary Disease

CR:

Partial remission (PR):

PD:

No response (NR):

Relapse:

A

CR: Complete resolution of lymphomatous enlargement by CT. For patients with a previous positive PET scan, a posttreatment residual mass of any size is considered a CR as long as it is PET negative.

Partial remission (PR): > 50% decrease in the sum of the product of the greatest perpendicular diameters (SPD) of the lymphomatous enlargement. For patients with a previous positive PET scan, post-treatment PET must be positive in at least one previously involved site.

PD: > 25% increase in the SPD of the lymphomatous enlargement. For patients with a previous positive PET scan, posttreatment PET must be positive in at least one previously involved site.

No response (NR): Does not meet criteria for either PR or PD.

Relapse: Recurrence of lymphomatous enlargement after achieving CR.

49
Q

The preferred sample for MRD assessment is:

A

First small volume (of up to 3 mL) pull of the bone marrow aspirate

50
Q

If MRD is negative by flow cytometry, an FDA-approved ___________________ should be considered to confirm negativity.

A

NGS assay

51
Q

Timing of MRD assessment:

A
  • Upon completion of initial induction
  • End of consolidation

  • Additional time points should be guided by the regimen used and risk features.
  • Serial monitoring frequency may be increased in patients with molecular relapse or persistent low-level disease burden.
  • For some techniques, a baseline sample (ie, prior to treatment) is needed to characterize the leukemic clone for subsequent MRD assessment.
52
Q

Risk factors for developing ALL include

A
  • Age >70 years
  • Exposure to chemotherapy or radiation therapy
  • Genetic disorders, particularly Down syndrome
53
Q

Other genetic conditions categorized as a risk factor for ALL

A
  • Li-Fraumeni syndrome
  • Neurofibromatosis
  • Klinefelter syndrome
  • Fanconi anemia
  • Shwachman-Diamond syndrome
  • Bloom syndrome
  • Ataxia telangiectasia
54
Q

Disease is restricted to a mass lesion primarily involving nodal or extranodal sites with no or minimal involvement in blood or bone marrow (generally defined as <20% lymphoblasts in the marrow)

A

Lymphoblastic lymphoma

Based on morphologic, genetic, and immunophenotypic features, lymphoblastic lymphoma is indistinguishable from ALL.

Patients with lymphoblastic lymphoma generally benefit from treatment with ALL-like regimens

55
Q

Immunophenotype of Early pre-B-ALL

A
  • Terminal deoxynucleotidyl transferase (TdT)
  • CD19/CD22/CD79a
  • Absence of CD10 (formerly termed common ALL antigen) or surface immunoglobulins

CD10 negativity correlates with KMT2A rearrangement and poor prognosis

56
Q

Immunophenotype of Pre-B-ALL

A
  • Cytoplasmic immunoglobulins
  • CD10/CD19/CD22/CD79a expression

Mature B-ALL shows positivity for surface immunoglobulins and clonal lambda or kappa light chains, and is negative for TdT.

57
Q

Immunophenotype of T-cell lineage ALL

A
  • Cytoplasmic CD3 (T-cell lineage blasts) or cell surface CD3 (mature T cells)
  • CD1a/CD2/CD5/CD7
  • TdT

CD52 may be expressed in 30% to 50% of T-cell lineage ALL in adults.

58
Q

Immunophenotype of T-cell lineage ETP-ALL

A
  • Absence of CD1a/CD8
  • Weak expression of CD5 (<75% positive lymphoblasts)
  • Presence of 1 or more myeloid or stem cell markers (CD117, CD34, HLA-DR, CD13, CD33, CD11b, or CD65) on at least 25% of lymphoblasts

Accounts for 12% of pediatric T-ALL (and about 2% of ALL), and is associated with poor clinical outcomes even with contemporary treatment regimens

59
Q

The most common chromosomal abnormality among children with ALL

A

Hyperdiploidy (>50 chromosomes) 25%

2nd: chromosomal translocation t(12;21) –> ETV6::RUNX1 subtype

Both hyperdiploidy and ETV6::RUNX1 subtypes are associated with favorable outcomes in ALL

60
Q

The most common chromosomal abnormality among adult with ALL

A

t(9;22)(q34;q11): Philadelphia chromosome (Ph)

BCR::ABL1

2nd: Ikaros–> IKZF1 25%–35%

61
Q

The NCCN Panel now considers Ph-positive ALL without IKZF1 plus and without antecedent chronic myeloid leukemia (CML) as ________________ risk

A

Standard risk

NCCN (13 decks)

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