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WILLIAM'S HEMATOLOGY > 87 Acute Myelogenous Leukemia > Flashcards

87 Acute Myelogenous Leukemia Flashcards

1
Q

4 environmental factors are established causal agents of AML

A
  • high-dose radiation exposure
  • chronic, high-dose benzene exposure (≥40 parts per million [ppm]-years)
  • chronic tobacco smoking
  • chemotherapeutic (DNA-damaging) agents
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2
Q

An endogenous factor that increases risk is obesity. Studies in North America show an increased risk of AML in men and women with elevated body mass index, and this is particularly notable for __________________

A

Acute promyelocytic leukemia (APL)

The precise mechanisms are still unclear but may be related, in part, to elevated leptin levels, decreased adiponectin levels, shortened telomeres, alterations in lipid metabolism, associated inflammation and as yet unknown factors in obese subjects

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3
Q

The most compelling data indicate that the bulk of AML cases arise from 1 of 2 predominant CD34+ cell populations:

A

CD34+CD45RA+CD38−CD90− (multipotential myeloid progenitor)

or

CD34+CD38+CD45RA+CD110+ (granulocyte–monocyte progenitor)

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4
Q

Chromosome changes involving CBF

A

t(8;21), inv(16), t(16;16), or t(15;17)

A more favorable outcome

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5
Q

Increased in frequency in patients over 60 years of age and in cases that develop after cytotoxic therapy

A

Deletions in chromosome 5 and 7 and complex cytogenetic abnormalities

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6
Q

Mutations with favorable outcomes

A
  • t(8;21)(q22;q22.1); RUNX1-RUNX1T1
  • inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFβ-MYH11
  • Mutated NPM1 without FLT3-ITD or with FLT3-ITDlow
  • Biallelic mutated CEBPa
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7
Q

Mutations with poor outcome

A
  • t(6;9)(p23;q34.1); DEK-NUP214
  • t(v;11q23.3); KMT2A rearranged
  • t(9;22)(q34.1;q11.2); BCR-ABL1
  • inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2,MECOM(EVI1)
  • −5 or del(5q); −7; −17/abn(17p)
  • Complex karyotype, monosomal karyotype
  • Wild-type NPM1 and FLT3-ITDhigh
  • Mutated RUNX1or ASXL1 without good risk karyotype
  • Mutated TP53
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8
Q

TRUE OR FALSE

Patients with CBF leukemias expressing KIT have a good prognosis.

A

FALSE

Patients with CBF leukemias expressing KIT have a worse prognosis.

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9
Q

TRUE OR FALSE

A monosomal karyotype is associated with a decreased chance of achieving remission or survival, especially when combined with TP53 mutations.

A

TRUE

A monosomal karyotype is associated with a decreased chance of achieving remission or survival, especially when combined with TP53 mutations.

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10
Q

Approximately _______of AML cases have a normal karyotype

A

45%

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11
Q

Most frequently mutated gene in AML (50%)

Allogenic transplantation not needed in first
remission if this mutation occurs in absence
of mutated FLT3-ITD

A

NPM1

NPM1 mutation is not associated with better duration of complete remission in those treated with hypomethylating agents.

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12
Q

An ITD of FLT3 on chromosome 13 occurs in approximately ____ of adult AML cases

A

25%

FLT3-ITD expression is often higher at relapse.

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13
Q

Point mutations in the TKD of FLT3 mutations occur in approximately ____% of AML cases

A

6%

Have little impact on outcomes

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14
Q

The FLT3-ITD mutation confers a poor prognosis if the ratio of mutant to wild-type expression is (LOW/HIGH).

A

High (≥0.51)

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15
Q

A leucine zipper transcription factor involved in myeloid differentiation.

A

CEBPα

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16
Q

TRUE OR FALSE

CEBPα-double, but not CEBPα-single, mutation patients had a significantly better overall survival at 8 years than wild-type, CEBPα-single, or CEBPα-double and FLT3-ITDpositive patients.

A

TRUE

CEBPα-double, but not CEBPα-single, mutation patients had a significantly better overall survival at 8 years than wild-type, CEBPα-single, or CEBPα-double and FLT3-ITDpositive patients.

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17
Q

Catalyze oxidative decarboxylation of isocitrate into α-hemoglutarate

A

IDHs

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18
Q

Found to predict for the presence of IDH1/IDH2 mutations

A

Serum 2hydroxyglutarate

  • A level of 700 ng/mL was found to discriminate mutated from nonmutated
  • Those with levels greater than 20 ng/mL at the time of remission had shorter overall survival.
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19
Q

These mutations are highly enriched in therapy-related AML and in those with complex karyotype.

A

TP53 Mutations

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20
Q

The detection of persistent leukemia-associated mutations in at least ______% of marrow cells in day 30 remission marrow cell samples is associated with a high risk of relapse.

A

5%

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21
Q

Gene mutations associated with familial AML

A
  • GATA2
  • CEBPα and DDX41
  • Telomerase RNA (TERC) or telomerase reverse transcriptase component (TERT)
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22
Q

AML is the predominant form of leukemia during the: (Neonatal OR childhood OR adolescence)period

A

Neonatal period

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23
Q

AML is more common in (males/females)

A

Males

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24
Q

The acute promyelocytic variant of AML is somewhat more common in __________ (race)

A

Latinos

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25
Q

Immunologic Phenotype

CD11b, CD13, CD15, CD33, CD117,
HLA-DR

A

Myeloblastic

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26
Q

Immunologic Phenotype

CD11b, CD13, CD14, CD15, CD32, CD33,
HLA-DR

A

Myelomonocytic

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27
Q

Immunologic Phenotype

Glycophorin, spectrin, ABH antigens,
carbonic anhydrase I, HLA-DR, CD71
(transferrin receptor)

A

Erythroid

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28
Q

Immunologic Phenotype

CD13, CD33

A

Promyelocytic

No CD34 and HLADR

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29
Q

Immunologic Phenotype

CD11b, 11c, CD13, CD14, CD33, CD65,
HLA-DR

A

Monocytic

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30
Q

Immunologic Phenotype

CD34, CD41, CD42, CD61, anti–von
Willebrand factor

A

Megakaryoblastic

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31
Q

Immunologic Phenotype

CD11b, CD13, CD33, CD123, CD203c

A

Basophilic

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32
Q

Immunologic Phenotype

CD13, CD33, CD117

A

Mast cell

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33
Q

Palpable splenomegaly or hepatomegaly occurs in approximately ______of patients.

A

25%

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34
Q

Lymphadenopathy is extremely uncommon, except in the ______________ variant of AML

A

Monocytic variant of AML

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35
Q

Extramedullary involvement is most common in____________________leukemia.

A

Monocytic or myelomonocytic leukemia

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36
Q

Skin involvement in AML may be of 3 types:

A
  • Nonspecific lesions
  • Leukemia cutis
  • Granulocytic (myeloid) sarcoma of skin and subcutis
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37
Q

A necrotizing inflammatory lesion involving the terminal ileum, cecum, and ascending colon, can be a presenting syndrome or occur during treatment

A

Ileotyphlitis (enterocolitis)

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38
Q

A tumor composed of myeloblasts, monoblasts, or megakaryocyes

A

Myeloid sarcoma

Synonyms: granulocytic sarcoma, chloroma, myeloblastoma, monocytoma

The tumors originally were called chloromas because of the green color imparted by the high concentration of the enzyme myeloperoxidase present in myelogenous leukemic cells.

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39
Q

Most frequent cytogenetic disturbance in myeloid sarcomas

A

Abnormalities in chromosome 8

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40
Q

(Systemic chemotherapy or local therapy), should be used for treatment of myeloid sarcoma, although the long-term outcome in such cases usually is poor.

A

Systemic chemotherapy

Systemic chemotherapy, rather than local therapy, should be used for treatment, although the long-term outcome in such cases usually is poor.

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41
Q

Mutations with propensity to develop extramedullary leukemia

A

AML with t(8;21) or inv16

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42
Q

Principal cause of anemia in AML

A

Inadequate production of red cells

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43
Q

Mechanism of thrombocytopenia in AML

A

Inadequate production and decreased survival of platelets

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44
Q

Are elliptical cytoplasmic inclusions, approximately 1.0–1.5 μm long and 0.5 μm wide, that derive from azurophilic granules

A

Auer rods

APL: higher proportion of cells have Auer rods and some have multiple (bundles) of rods (so-called faggot cells).

Present in the blast cells of approximately 15% of cases

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45
Q

The WHO has invoked an arbitrary threshold of______of marrow nucleated cells being blast cells to distinguish polyblastic AML from oligoblastic myelogenous leukemia

A

20%

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46
Q

Relapse of AML can be identified as any increase in blast count greater than _______

A

2%

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47
Q

TRUE OR FALSE

Any distinctions between MDS and AML in survival are a function of age, cytogenetic risk category, and molecular features, and the blast count.

A

FALSE

Any distinctions between MDS and AML in survival are a function of age, cytogenetic risk category, and molecular features, not the blast count.

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48
Q

Myeloblasts are distinguished from lymphoblasts by any of 3 pathognomonic features:

A
  • Reactivity with specific histochemical stains
  • Auer rods in the cells
  • Reactivity with a panel of monoclonal antibodies against epitopes present on myeloblasts (eg, CD13, CD33, CD117)
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49
Q

Leukemic myeloblasts give positive histochemical reactions for:

A

Myeloperoxidase, Sudan black B, or naphthyl AS-D-chloroacetate esterase

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50
Q

Blast cells may express :

Granulocytic surface antigens __________________ or
Monocytic surface antigens _____________

A

Granulocytic surface antigens (CD15, CD65) or
Monocytic surface antigens (CD11b, CD11c, CD14, CD64)

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51
Q

AML associated with intense fibrosis

A

Megakaryoblastic leukemia

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52
Q

Associated with marrow basophilia

A

t(6;9)

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53
Q

Associated with marrow eosinophilia

A

inv16 or t(16;16)

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54
Q

Associated with in cases of AML following chemotherapy or radiotherapy

A

Loss of part or all of chromosomes 5 and 7

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55
Q

Chromosome abnormality very common in acute myeloblastic leukemia

A

Trisomy 8

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56
Q

t(9;22) (q34; q22) in BCR-ABL1 gene is present in ______ of patients with AML

A

~2%

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57
Q

Often acute myelomonocytic phenotype; associated with increased marrow
eosinophils; predisposition to cervical lymphadenopathy, better response to
therapy

A

Inv(16) (p13.1;q22) or
t(16;16) (p13.1;q22)

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58
Q

~1% of cases of AML

Approximately 85% of cases with normal or increased platelet count

Marrow has increased dysmorphic, hypolobulated megakaryocytes.

Hepatosplenomegaly more frequent

A

Inv(3) (q21q26.2) /RPN1-EVI1

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59
Q

Approximately ______% of cases of AML contain cells that are cytogenetically normal.

A

45%

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60
Q

Serum uric acid and lactic dehydrogenase levels are higher in___________________AML than in other AML phenotypes

A

Myelomonocytic and monocytic AML

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61
Q

Are associated with hypofibrinogenemia and other indicators of activation of coagulation or fibrinolysis

A

APL and acute monocytic leukemia

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62
Q

Hyperleukocytosis is a markedly elevated blood blast cell count, usually greater than _____________

A

100 × 10 9 /L

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63
Q

Subsets of AML are associated with a greater likelihood of presenting with hyperleukocytosis

A

Myelomonocytic, acute monocytic, the microgranular variant of APL, and AML with inv16, 11q23 rearrangements, or FLT3-ITD

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64
Q

The circulations most sensitive to the effects of leukostasis

A

CNS, lungs, and penis

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65
Q

Approximately 10% of patients with AML present with a syndrome that includes pancytopenia, often with inapparent blood blast cells, and absence of hepatic, splenic, or lymph nodal enlargement

A

Hypoplastic Leukemia

Approximately 75% of these patients are men older than 50 years.

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66
Q

Marrow necrosis is uncommon but approximately _________ of cases are associated with lymphoid or myeloid malignancies and about ____________ of cases occur in patients with AML

A

two-thirds

one-fourth

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67
Q

Two most common symptoms or signs of marrow necrosis

A

Bone pain (approximately 80% of patients)

Fever (approximately 70% of patients)

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68
Q

Marrow findings in marrow necrosis

A

The marrow aspirate is often watery and serosanguineous.

An amorphous extracellular eosinophilic background with disintegrating cells that have lost their staining characteristics with indistinct margins and varying degrees of pyknosis and karyorrhexis

Both technetium scanning and MRI can point to areas of intact marrow that may be used to make a diagnosis of the underlying disease, if it is unknown.

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69
Q

Four myeloproliferative syndromes related to AML have been identified in the neonate:

A
  • Transient myeloproliferative disorder
  • Transient leukemia
  • Congenital leukemia
  • Neonatal leukemia

Transient myeloproliferative disorder and transient leukemia are considered to represent the same phenomenon.

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70
Q

Can be present at birth, or occur shortly thereafter, in approximately 10% of infants with Down syndrome.

The blast cells usually have the immunophenotype of megakaryocytes.

A

Transient myeloproliferative disease (TMD)

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71
Q

In Transient myeloproliferative disease (TMD), the elevated white cell and blast cell counts disappear in most patients (approximately 80%) over a period of _____________

A

Weeks to months

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72
Q

Approximately _______of newborns with Down syndrome and transient leukemia develop______________________ in the first __________years of life.

A

25%

Acute megakaryocytic leukemia

First 4 years of life

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73
Q

Mutations that have been found in nearly all patients with TMD and in acute megakaryocytic leukemia in Down patients

A

GATA1 mutations

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74
Q

TMD

Treatment suggested for those patients with severe hepatic fibrosis, very high white cell counts, or hydrops fetalis

A

Very-low-dose cytarabine

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75
Q

TMD

Children with Down syndrome have either a ______-fold risk of AML or an approximately ________-fold of ALL by age 5 years

A

150x (AML)

40x (ALL)

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76
Q

Myelogenous leukemia in patients with Down syndrome often has what phenotype of leukemia

A

Megakaryoblastic or erythroid phenotype

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77
Q

A rare syndrome, occurs 10 times more often in newborns with Down syndrome than in newborns without trisomy 21

The disease has been diagnosed prenatally.

A

Congenital or neonatal leukemia

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78
Q

The most common phenotype and karyotype in congenital or neonatal leukemia

A

Monocytic leukemia and t(4;11)

The presence of a cytogenetic abnormality on band q23 of chromosome 11 is a very poor prognostic sign.

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79
Q

Cases of biphenotypic leukemia that are morphologically or cytochemically indicative of myelogenous leukemia

A

LY+AML

The WHO now classifies some of these disorders as mixed phenotype acute leukemia (MPAL).

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80
Q

Cases of biphenotypic leukemia that are more indicative of lymphocytic leukemia

A

MY+ALL

The WHO now classifies some of these disorders as mixed phenotype acute leukemia (MPAL).

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81
Q

2 notable syndromes are associated with hybrid leukemias:

A

(a) the myeloid leukemia and natural killer cell hybrid (CD56+, CD7+, CD13+, CD33+)

(b) the lymphoma, eosinophilia, and t(8;13) myeloid leukemia hybrid.

  • The hybrids can appear de novo or after relapse of a lymphoma, T-cell leukemia, or blast crisis of CML.
  • The hybrid leukemias usually have a poor prognosis.
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82
Q

Often simulates APL, with hypergranular cytoplasm present but an abnormality of chromosome 17 is absent

A

Myeloid–natural killer cell leukemia

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83
Q

Lymphoid and myeloid cells are present simultaneously but are derived from separate clones, or sequential myeloid and lymphoid leukemia are present, but the 2 lineages are derived from separate clones

A

Mixed Leukemias

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84
Q

An unusual but significant concordance has been reported between nonseminomatous mediastinal germ cell tumors and AML, especially the _________________ variant.

A

Megakaryoblastic

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85
Q

Most common in adults, and most frequent variety in infants.

Three morphologic– cytochemical types (M0, M1, M2).

A

Acute myeloblastic leukemia

The WHO has divided acute myeloblastic leukemia not otherwise specified, into 3 types: AML without differentiation, AML without maturation, and AML with maturation.

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86
Q

Approximately 15% of patients with AML

More likely to have extramedullary infiltrates in gingiva, skin, or CNS than are patients with acute myeloblastic leukemia

A

Acute Myelomonocytic Leukemia

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87
Q

FAB Classification of Acute Myelomonocytic Leukemia

Variant of myelomonocytic leukemia has increased numbers of marrow eosinophils (10–50%), Auer rods in blast cells, and inversion or rearrangement of chromosome 16

Has an increased risk of CNS involvement, it carries a more favorable prognosis

A

M4Eo

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88
Q

Translocations involving ______________ are associated with Acute Myelomonocytic Leukemia

A

Chromosome 3

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89
Q

Erythroid leukemia is arbitrarily divided into 3 degrees of severity:

A

(a) Erythroleukemia in which more than 50% of the marrow cells are dysmorphic

(b) Erythroblasts admixed with myeloblasts, the latter composing approximately 20% of nonerythroid cells or approximately 5% to 10% of total marrow cells

(c) Pure erythroid leukemia, in which more than 80% of marrow cells are dysmorphic erythroblasts with a trivial granulocytic proportion of cells and very few if any myeloblasts

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90
Q

Features of erythroblasts in Acute Erythroid leukemia

A

Glycophorin A, spectrin, carbonic anhydrase I, ABH blood group antigens, and other antigens that occur on early erythroid progenitors, such as the transferrin receptor (CD71)

Antihemoglobin antibody and antihuman erythroleukemic cell line antibody often are positive

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91
Q

TRUE OR FALSE

The more predominant the erythroid component and the lower the proportion of myeloblasts, the better the response to therapy.

A

TRUE

The more predominant the erythroid component and the lower the proportion of myeloblasts, the better the response to therapy.

92
Q

APL occurs with greater frequency among (nationality)

A

Latinos from Europe and South and Central America

93
Q

APL is also increased among persons with an (increased or decreased) body mass index.

A

Increased

Upregulation of polyunsaturated fatty acid metabolism genes has been noted, and in APL patients with obesity, FLT3 mutations are higher.

94
Q

Promyelocytes with multiple Auer rods have been referred to as:

A

Faggot cells

95
Q

Leukemic promyelocytes stain intensely with myeloperoxidase and Sudan black and express

A

CD9, CD13, and CD33

BUT NOT CD34 or HLA-DR

96
Q

Microgranular cases represent approximately _____ of patients with promyelocytic leukemia.

The leukemic cells may mimic promonocytes with convoluted or lobulated nuclei.

A

20%

The total WBC often is highly elevated, and severe coagulopathy is prominent in microgranular cases.

97
Q

The most frequent cytogenetic abnormality (>95%) in APL

A

t(15;17)(q22;q21)

98
Q

Other variant translocations in APL

A

Chromosome 3, 5, or 11 and chromosome 17 or isochromosome 17

99
Q

Rearrangement of the RARα gene that is retinoid responsive

A

t(15;17), PML–RARα fusion
t(5:17), NPM–RARα fusion
t(3;17), TBLR1–RARα fusion

100
Q

Rearrangement of the RARα gene that is retinoid resistant

A

t(11;17), PLZF–RARα fusion

101
Q

Mutation in APL where Auer rods are absent and CD56 expression usually is present

A

t(11;17)

102
Q

The PML–RARα gene has 2 isoforms that produce a short-type and a long-type fusion mRNA, respectively.

Patients with the__________ isoform may have a worse outcome

A

Short

FLT3 mutations are frequently found in human disease, especially in the hypogranular variant.

103
Q

APL

Approximately 5% to 10% of patients die during remission induction, most of

A

Hemorrhage
often into the brain

104
Q

Have a higher prevalence (50%) of extramedullary tumors

Hepatomegaly, splenomegaly, and lymphadenopathy are more frequent

A

Acute Monocytic Leukemia

105
Q

In Acute Monocytic Leukemia, monocytic cells react for nonspecific esterase stains, α-naphthyl acetate esterase, and naphthol AS-D-chloroacetate-esterase; in a cytochemical or chemoluminescence assay; or with monoclonal antibodies against monocyte surface antigens, especially ____

A

CD14

Immunoreactivity of cells for lysozyme is characteristic.

106
Q

Results in the MOZ-CBP fusion gene, is characterized by mildly granular promonocytes (simulating hypogranular promyelocytes), intense phagocytosis of red cells, erythroblasts, and sometimes neutrophils and platelets in blood and marrow, simulating macrophagic hemophagocytic syndrome, intravascular coagulation or primary fibrinolysis, and a high frequency of extramedullary disease

A

Acute monoblastic leukemia with t(8;16)

107
Q

TRUE OR FALSE

In acute monocytic leukemia, , examination of cerebrospinal fluid is often recommended, even in the absence of symptoms, when remission has been achieved

A

TRUE

In acute monocytic leukemia, examination of cerebrospinal fluid is often recommended, even in the absence of symptoms, when remission has been achieved

Greater incidence of CNS or meningeal disease

108
Q

AML prevalent variant of AML that develops in patients with Down syndrome or in patients with mediastinal germ cell tumors and coincident AML

Has severe myelofibrosis

The serum lactic acid dehydrogenase level frequently is strikingly increased and has an isomorphic pattern unlike that seen with other acute leukemias.

A

Acute Megakaryoblastic (Megakaryocytic) Leukemia

Maturing megakaryocytes with agranular cytoplasm with cytoplasmic protrusions, clusters of platelet-like structures, or shedding of cytoplasmic blebs

109
Q

Markers of Acute Megakaryoblastic (Megakaryocytic) Leukemia

A

Antibodies to von Willebrand factor or to platelet glycoprotein Ib (CD42), IIb/IIIa (CD41), or IIIa (CD61)

Confirmation of their megakaryoblastic maturation requires immunocytologic studies for the presence of von Willebrand factor and the immunoreactivity to CD41, CD42, or CD61.

110
Q

Chromosal abnormalities associated with Acute megakaryocytic leukemia

A

chromosome 3
Infants with t(1;22)(p13;q13)

111
Q

The eosinophilic cells are dysmorphic and the cytoplasm hypogranulated with smaller-than-normal eosinophilic granules.

A

Acute Eosinophilic Leukemia

Increased eosinophils in the marrow, but not in the blood, is a variant of acute myelomonocytic leukemia and inversion 16 or other abnormalities of chromosome 16, but is not considered an acute eosinophilic leukemia.

112
Q

Acute eosinophilic leukemia

A specific histochemical reaction, _______________________, permits identification of leukemic cells with eosinophilic differentiation and diagnosis of acute eosinoblastic leukemia in some cases of AML with fewer identifiable eosinophils in blood or marrow.

A

Cyanide-resistant peroxidase

113
Q

Proposed as a means of distinguishing acute eosinophilic leukemia from a polyclonal, reactive eosinophilia

A

Overexpression of WT gene expression

114
Q

TRUE OR FALSE

Patients with acute eosinophilic leukemia do not usually develop bronchospastic signs, neurologic signs, and heart failure from endomyocardial fibrosis as is seen in chronic eosinophilic leukemia

A

TRUE

Patients with acute eosinophilic leukemia do not usually develop bronchospastic signs, neurologic signs, and heart failure from endomyocardial fibrosis as is seen in chronic eosinophilic leukemia

Probably because those tissue changes are the result of release of toxins in the granule crystalloid, absent in most eosinophils in acute eosinophilic leukemia and because of the shorter duration of survival in acute eosinophilic leukemia.

115
Q

Appropriate choice for treatment for Acute eosinophilic leukemia

A

Cytarabine and an anthracycline

116
Q

Most cases evolve from the chronic phase of CML

Characterized by presence of CD9, CD25, or both

Elevated blood and urine histamine and urinary methylhistamine levels

A

Acute basophilic leukemia

In some cases of acute myelomonocytic leukemia associated with t(6;9)(p23;q34), basophils may be increased in the marrow, but not in the blood.

117
Q

Acute basophilic leukemia

The cells stain with ____________ and the basophilic granules can be most striking in myelocytes.

A

Toluidine blue or Astra blue

118
Q

Related to a mutation of the KIT gene

Plasma tryptase is elevated

Naphthol AS-D-chloracetate-esterase–positive, CD11b-negative, CD117-positive, CD123-negative

A

Mast cell leukemia

119
Q

The key laboratory distinctions between acute basophilic leukemia and acute mast cell leukemia

A

Basophilic leukemia: naphthol AS-Dchloracetate- esterase–negative, CD11b positive, CD117 negative or weakly positive, CD123 positive, have no increase in cell or plasma tryptase

Mast cell leukemia: naphthol AS-D-chloracetate-esterase–positive, CD11b-negative, CD117-positive, CD123-negative, have an increase in cell and plasma tryptase

120
Q

Defined as elimination of the leukemic cell population in marrow as judged by microscopy and flow cytometry and the restitution of marrow hematopoiesis resulting in a normal or virtually normal white cell, hemoglobin, and platelet concentrations in the blood

A

Remission

121
Q

Any suspicion of APL, however, should lead to urgent administration of ________________

A

ATRA

122
Q

TRUE OR FALSE

Age is a contraindication to treatment, and septuagenarians and octogenarians who are fit cannot enter remissions.

A

FALSE

Age per se is not a contraindication to treatment, and septuagenarians and octogenarians who are fit can enter remissions.

123
Q

More extensive evaluation of coagulation factors should be made if:

A

(a) clotting times are abnormal

(b) bleeding is exaggerated for the level of the platelet count

(c) APL or acute monocytic leukemia is the phenotype

124
Q

Therapy for hyperuricemia is required if:

A

(a) the pretreatment uric acid level is greater than 7 mg/dL (0.4 mmol/L)
(b) the marrow is packed with blast cells, or
(c) the blood blast cell count is moderately or markedly elevated

125
Q

Dose of Allopurinol

A

Allopurinol 300 mg/day

126
Q

Allopurinol can cause allergic dermatitis and should not be used if the uric acid level is less than_________ and the total white cell count is less than approximately _______, as long as hydration is adequate and urine flow is high (>_______ mL/h)

A

less than 7 mg/dL

total white cell count is less than approximately 20 × 109/L

> 150 mL/h

Thus, allopurinol should be discontinued after the risk of acute hyperuricosuria or tumor lysis has passed (usually 4 to 7 days).

127
Q

Reduce plasma urate levels by approximately 80% within 4 hours of the first drug dose.

Recommended dose is 0.2 mg/kg daily for 5–7 days IV

A

Recombinant urate oxidase (rasburicase)

128
Q

The primary goal of induction therapy in AML

A

Complete remission
* <2% blasts in the marrow)
* A neutrophil count greater than 1 × 109/L
* Platelet count greater than 100 × 109/L

129
Q

Current standard induction treatment for non-APL AML

A

Anthracycline or Anthraquinone and Cytarabine

130
Q

The two most important variables in remission-induction therapy

A
  • Age of the patients
  • Proportion of patients with therapy-induced leukemia or an antecedent clonal myeloid disease (clonal evolution)
131
Q

Anthracycline that does not induce P-glycoprotein expression -> drug resistance is reduced

A

Idarubicin

132
Q

May be given during induction to reduce the risk of cardiotoxicity in patients at higher-than-usual risk because of a history of coronary artery disease or congestive heart failure

A

Dexrazoxane

133
Q

TRUE OR FALSE

High-dose cytarabine does not increase complete remission rates and increases toxicity compared to conventional doses, especially in patients over 60 years of age

A

TRUE

High-dose cytarabine does not increase complete remission rates and increases toxicity compared to conventional doses, especially in patients over 60 years of age

134
Q

The effect of induction chemotherapy is usually assessed by marrow aspirate and biopsy _______days after completion of chemotherapy.

A

7–10 days

“day 14 marrow”

Hypocellular marrow and no evidence of residual leukemic blasts: recovery of normal counts is awaited

Hypocellular marrow and a small number of residual blasts: additional therapy may be delayed until count recovery or until another marrow assessment often recommended at a 1 week interval after the “day 14 marrow” examination.

Sgnificant amounts of leukemic cells remaining: repeating the original induction therapy or use of a high-dose cytarabine regimen

135
Q

TRUE OR FALSE

There are insufficient data to determine whether use of the same regimen or advancing to a high-dose cytarabine-containing regimen is the superior approach.

A

TRUE

There are insufficient data to determine whether use of the same regimen or advancing to a high-dose cytarabine-containing regimen is the superior approach.

136
Q

Approximately ____ of patients with persistent AML after 1 course of induction therapy have a complete remission after a second course, and disease-free survival at 5 years is approximately ___

A

40%

10%

137
Q

A multitargeted kinase inhibitor, was approved by the FDA in 2017 for use in AML patients with FLT3 ITD or TKD point mutations

A

Midostaurin (ITD or TKD)

Sorafenib (ITD)

138
Q

Second-generation FLT3 inhibitor

A

Gilteritinib and crenolanib

139
Q

A humanized anti-CD33 monoclonal antibody conjugated to calicheamicin that has now been approved for addition to 7+3 chemotherapy

A

Gemtuzumab ozogamicin (GO)

3 mg/m2 on days 1, 4, and 7

140
Q

The main toxicity of Gemtuzumab ozogamicin (GO)

A

Prolonged thrombocytopenia

141
Q

A liposomal preparation of daunorubicin hydrochloride and cytarabine liposome in a fixed 1:5 molar ratio, was approved for use by the FDA in 2017 for patients over 60 years with AML and in those with secondary AML or MDS-related cytologic findings

A

CPX-351

Toxicity: longer duration of cytopenia

142
Q

Use has been primarily in patients over 60 years deemed unfit for standard induction chemotherapy

A

Hypomethylating Agents

Decitabine
5-Azacytidine

143
Q

Oral bioavailable small molecule inhibitor of BCL-2

Demonstrated synergistic activity with hypomethylating agents

Approved in the United States for use in previously untreated AML patients older than 75 years in combination with low-intensity chemotherapy,

A

Venetoclax

Mechanisms of resistance to venetoclax include overexpression of BCL-2A1, BCL-xL, and MCL-1,738 as well as through alterations in metabolic pathways.

144
Q

A hedgehog pathway inhibitor approved by the FDA in 2018 for oral administration in combination with low-dose cytarabine for newly diagnosed AML patients 75 years old and older or who have comorbidities that preclude use of intensive induction chemotherapy

A

Glasdegib

145
Q

Toxicity of Glasdegib

A

Muscle cramps, dysgeusia, and prolonged QT interval

146
Q

Oral inhibitor of IDH2:
Oral inhibitor of IDH1:

A

Oral inhibitor of IDH2: Enasidenib
Oral inhibitor of IDH1: Ivosidenib

Only ivosidenib has been approved for initial therapy

147
Q

Side effects of hypomethylating agents

A

Constipation

148
Q

Side effect of Ivosidenib

A

Differentiation syndrome

149
Q

Most serious complications of hyperleukocytosis:

A

Intracranial hemorrhage or pulmonary insufficiency

150
Q

In management of hyperleukocytosis: - Hydration should be administered promptly to maintain urine flow greater than ____________

A

100 mL/h per m2

151
Q

Hydroxyurea dose for Cytoreduction

A

Hydroxyurea 1.5–2.5 g orally every 6 hours (total dose 6–10 g/day) for approximately 36 hours

Appropriate remission-induction therapy should be initiated as soon as possible after the leukocyte count has been decreased significantly.

152
Q

Simultaneous_____________________ can decrease blast cell concentration by approximately _______ within several hours without contributing to uric acid or cellular phosphate release.

A

Leukapheresis

30%

153
Q

____________________ may improve the hypoxemia related to hyperleukocytosis

A

Inhaled nitric oxide

154
Q

Granulocyte transfusion should not be used prophylactically for neutropenia but may be used in patients with :

A
  • high fever, rigors, and bacteremia unresponsive to antibiotics
  • with blood fungal infections
  • with septic shock
155
Q

TRUE OR FALSE

Patients with evidence of intravascular coagulation or exaggerated primary fibrinolysis should be considered for platelet and fresh-frozen plasma administration before antileukemic therapy is started.

A

TRUE

Patients with evidence of intravascular coagulation or exaggerated primary fibrinolysis should be considered for platelet and fresh-frozen plasma administration before antileukemic therapy is started.

156
Q

Infusion of cryoprecipitate can be used for fibrinogen levels less than approximately ____________

A

125 mg/dL

157
Q

Intravascular coagulation or primary fibrinolysis may occur in patients with _____________

A

APL and acute monocytic leukemia

158
Q

Management of CNS disease

Prophylactic therapy usually is not indicated, but examination of the spinal fluid after remission should be considered in:

A

(a) monocytic subtypes;
(b) cases with extramedullary disease;
(c) cases with inversion 16 and t(8;21) cytogenetics;
(d) CD7+ and CD56+ (neural cell adhesion molecule) immunophenotypes; and
(e) patients who present with very high blood blast cell counts

159
Q

TRUE OR FALSE

Prophylactic intrathecal chemotherapy is not recommended if low-dose cytarabine is used for consolidation.

A

FALSE

Prophylactic intrathecal chemotherapy is NOT recommended if high-dose cytarabine is used for consolidation.

160
Q

Treatment of meningeal leukemia

A
  • High-dose IV cytarabine (which penetrates the blood–brain barrier)
  • Intrathecal methotrexate
  • Intrathecal cytarabine
  • Cranial radiation
  • Chemotherapy and radiation in combination
161
Q

If CNS leukemia is present, intrathecal therapy is often given:

A

Twice per week until blasts are cleared, and then once per week for 4–6 weeks

This therapy can be accomplished via the lumbar puncture route or through placement of an Ommaya reservoir.

162
Q

If there is a mass present treatment can be:

A

Radiation or high-dose cytarabine with glucocorticoids

163
Q

TRUE OR FALSE

Unless the patient has neurologic symptoms, lumbar puncture generally is deferred until blood blast cells have cleared.

A

TRUE

Unless the patient has neurologic symptoms, lumbar puncture generally is deferred until blood blast cells have cleared.

No consensus exists on a trigger for platelet transfusion in adults with AML undergoing lumbar puncture; a platelet count of less than 20 × 109/L has been proposed as such a trigger, but many therapists use a higher platelet count (eg, 50 × 109/L) as a safety threshold for lumbar puncture.

164
Q

Myeloid sarcoma may be the presenting finding in approximately ____ of patients with AML.

A

1%

Patients with trisomy 8 have poorer survival rates.

165
Q

Treatment for Nonleukemic Myeloid Sarcoma

A

Intensive AML induction therapy

166
Q

Post remission therapy for patients with good risk cytogenetics and can also be considered in those with intermediate-risk cytogenetics

A

4 cycles of high-dose cytarabine

167
Q

Post remission therapy for patients with poor-risk cytogenetics

A

Allogeneic HSC transplantation

168
Q

Associated with benefit from high-dose cytarabine therapy:

A cycle is typically 3 g/m2 twice daily on days 1, 3, and 5, providing 6 doses per cycle

A
  • RAS mutations
  • CBF leukemias, such as t(8;21)
  • Patients 60 years or younger, if they have adequate renal function
  • NPM1 mutation without FLT3-ITD mutation
  • Biallelic CEBPα mutations

KIT mutations in CBF AML are associated with a poorer prognosis.

169
Q

High-dose cytarabine frequently causes conjunctivitis and photophobia hence this should be given

A

Glucocorticoid eyedrops are usually used every 6 hours for 24–72 hours after the last dose of the drug

170
Q

May decrease the likelihood of severe cerebellar toxicity associated with Cytarabine

A

1-hour duration infusion of high-dose or reduced-dose (eg, 2 g/m2) cytarabine

Patients over age 60 years and patients with renal insufficiency require dose attenuation (ie, to 1–2 g/m2).

171
Q

Can be used for AML who achieve a remission, do not have a compatible stem cell donor, and are into the eighth decade of life

A

Autologous marrow or blood stem cell rescue

172
Q

An adoptive immunotherapy with donor mononuclear cell infusions is sometimes used to treat relapse of leukemia after allografting

A

Donor Leukocyte Infusion

Most effective in early relapses and in the absence of extensive of chronic GVHD.

173
Q

The major complications of Donor Leukocyte Infusion

A

GVHD and marrow aplasia

174
Q

After patients complete consolidation therapy, they are generally followed with blood counts every _______________

A

Every 3 months for 2 years, and then every 3–6 months for 5 years

175
Q

A predictive factor for relapse

A

Time taken to enter complete remission after induction

176
Q

TRUE OR FALSE

In patients who relapse more than 1 year after the first remission, the original remission-induction regimen can be readministered or a combination salvage chemotherapy regimen can be administered.

A

TRUE

In patients who relapse more than 1 year after the first remission, the original remission-induction regimen can be readministered or a combination salvage chemotherapy regimen can be administered.

177
Q

Defined as leukemia that does not respond to 2 initial induction chemotherapy treatments with cytarabine and an anthracycline or anthraquinone.

A

Refractory leukemia

178
Q

TRUE OR FALSE

A retrospective study suggested that clofarabine-based versus fludarabine-based regimens had a higher complete remission rate and a longer survival

A

TRUE

A retrospective study suggested that clofarabine-based versus fludarabine-based regimens had a higher complete remission rate and a longer survival

179
Q

Leukemia that reoccurs following a remission

A

Relapsed leukemia

180
Q

The probability of a second remission is approximately _% in younger (ages 15–60 years) patients, and approximately _% in older (ages 60–80 years) patients, but the duration of remission is nearly always much shorter than the first remission.

A

40%

25%

181
Q

Second-generation DNA methylation inhibitors

A

Guadecitabine

182
Q

Can promote histone acetylation and gene transcription in RUNX1/ETO-positive leukemic cells

A

Depsipeptide

183
Q

An aminonucleoside inhibitor of DOT1L histone methyltransferase activity is under clinical investigation in MLL-rearranged leukemias.

A

Pinometostat

184
Q

Are small molecule inhibitors of MDM2 (Murine Double Minute 2), a negative regulator of p53

A

Nutlins / Idasanutlin

185
Q

An oral exportin-1 inhibitor

A

Selinexor

186
Q

A CDK6 inhibitor has activity in MLL-rearranged leukemias

A

Palbociclib

187
Q

A CDK9 inhibitor, is being examined as an MCL-1 inhibitor

A

Alvocidib

188
Q

Has been studied in CBF leukemias with a KIT mutation in conjunction with chemotherapy

A

Dasatinib (Sprycel)

189
Q

Inhibitor of oxidative phosphorylation

A

Metformin
Mubritinib

190
Q

An oral iron chelator, inhibits leukemic CD34+CD38− cells through NF-κB inhibition and reactive oxygen species generation

A

Deferasirox (Exjade)

191
Q

TRUE OR FALSE

Patients who are suspected APL based on morphology and presence of coagulopathy should begin ATRA without waiting for definitive FISH or molecular confirmation.

A

TRUE

Patients who are suspected APL based on morphology and presence of coagulopathy should begin ATRA without waiting for definitive FISH or molecular confirmation.

192
Q

In APL, those with WBC of ________________ or higher are considered to have high-risk disease

A

10 × 109/L

193
Q

Used alone, ATRA can induce a short-term remission in at least _______ of patients.

A

80%

194
Q

Leukocytosis during ATRA–arsenic trioxide therapy in patients with white cell counts higher than 10 × 109/L can be treated with _____________.

A

Hydroxyurea or idarubicin

195
Q

Chemotherapy for APL

Low-risk disease:

High-risk patients:

A

Chemotherapy for APL

Low-risk disease: A combination of ATRA plus arsenic trioxide, ATRA plus idarubicin alone, or ATRA plus daunorubicin plus cytarabine

High-risk patients: ATRA plus daunorubicin and cytarabine, ATRA plus idarubicin, or ATRA and arsenic trioxide with idarubicin (dose-adjusted based on age)

196
Q

Cause of mortality for APL despite treatment with ATRA

A

Fatal intracranial hemorrhage

5%

High white count has been found to be a predictor of early hemorrhagic death.

197
Q

Variant of APL, in which the promyelocytic leukemia zinc finger (PLZF) gene is fused to RARα, DOES NOT respond to ATRA.

A

t(11;17)

198
Q

With ATRA, leukemic promyelocytes disappear from the blood in ______weeks, and a normal marrow aspirate maybe obtained in ______ weeks.

A

2–4 weeks

4–10 weeks

199
Q

A rapid increase in the total blood leukocyte count to as high as 80 × 109/L in the first several weeks of therapy of APL

A

Differentiation syndrome

(previously called the retinoic acid syndrome)

200
Q

Key feature or symptom of Differentiation Syndrome

A

Respiratory distress

The syndrome consists of fever, weight gain, dependent edema, pleural or pericardial effusion, and bouts of hypotension.

201
Q

Treatment of differentiation syndrome

A

Early use of cytotoxic chemotherapy and glucocorticoid administration

Dexamethasone 10 mg IV every 12 hours

202
Q

Prophylactic glucocorticoids should be given to those:

A
  • With a WBC higher than 10 × 109/L or
  • Those receiving both ATRA and arsenic trioxide
203
Q

APL

Targeted levels for
Platelet counts:
Fibrinogen:

A

Platelet counts: 30–50 × 109/L
Fibrinogen: 1.5 g/L or higher

204
Q

TRUE OR FALSE

Arsenic trioxide can trigger apoptosis of APL cells at low concentrations and maturation at high concentrations.

A

FALSE

Arsenic trioxide can trigger apoptosis of APL cells at high concentrations and maturation at low concentrations.

205
Q

Consolidation therapy for APL

A

Arsenic trioxide plus ATRA or an anthracycline plus ATRA

High Risk: the addition of cytarabine or use of arsenic trioxide can be used to diminish the rate of relapse

High-risk patients may require intrathecal chemotherapy during consolidation to prevent central nervous system relapse.

206
Q

At the end of consolidation, molecular remission in the marrow should be assessed by

A

Reverse transcriptase–polymerase chain reaction (RT-PCR)

207
Q

TRUE OR FALSE

In those patients treated with ATRA plus arsenic trioxide, and white cell count lower than 10 × 109/L at diagnosis, no maintenance is required.

A

TRUE

In those patients treated with ATRA plus arsenic trioxide, and white cell count lower than 10 × 109/L at diagnosis, no maintenance is required.

For others, ATRA maintenance with chemotherapy is recommended

Best results were achieved when ATRA was combined with 6-mercaptopurine and methotrexate.

208
Q

APL maintenance is usually recommended for ______ years.

A

2 years

During maintenance, PCR monitoring on blood samples is recommended.

If the PCR is positive in blood, a marrow examination should be done.

209
Q

APL High Risk

MRD monitoring should be performed every ___ months in high-risk patients up to ___ years after completion of consolidation therapy.

A

3 months

3 years

210
Q

TRUE OR FALSE

Secondary AML responds more poorly to chemotherapy and allogeneic HSC transplantation than does de novo AML.

A

TRUE
‘
Secondary AML responds more poorly to chemotherapy and allogeneic HSC transplantation than does de novo AML.

211
Q

Exposure to wha drugs can lead to AML with MLL gene rearrangements on chromosome 11q32.

A

Topoisomerase II inhibitors (eg, etoposide, mitoxantrone, amsacrine)

212
Q

Latency period for development of AML after:

Topoisomerase II Inhibitors :
Alkylating Agents and Cisplatin :

A

Topoisomerase II Inhibitors : 2 years
Alkylating Agents and Cisplatin : 6 years

Topoisomerase II Inhibitors : No relationship with higher cumulative dose has been identified.

Alkylating Agents and Cisplatin : The risk is related to cumulative alkylating agent dose.

213
Q

The most common cytogenetic changes in secondary AML from Alkylating Agents and Cisplatin

A

Deletions of all or part of chromosome 5 or 7

214
Q

Other drugs that may increase the risk of secondary leukemias:

A
  • Low-dose weekly methotrexate for rheumatoid arthritis
  • Etanercept therapy
  • Temozolamide
  • Growth hormone administration
  • G-CSF given to patients with congenital, but not idiopathic or cyclic neutropenia
215
Q

Characteristics of AML older than 60 years old

A
  • More frequent CD34 expression
  • High frequency of unfavorable cytogenetic findings (32%)
  • Higher MDR1 expression (71%)
  • Functional drug efflux (58%)
216
Q

Leukemia (AML, ALL, CML) is the _________ most common malignancy of women in the childbearing age group

A

Second

217
Q

AML in Pregnancy

If the pregnancy is not terminated,_______________ might be useful in the__________________ trimester, when chemotherapy poses a high risk to the embryo.

A

Leukapheresis

First

218
Q

The preferred anthracycline to treat pregnant women as it has lower transplacental transfer

A

Doxorubicin

219
Q

TRUE OR FALSE

Leukemic infiltrates can be found on both the maternal side of the placenta and the villi.

A

FALSE

Leukemic infiltrates can be found on the maternal side of the placenta, but usually not in the villi.

220
Q

The risk of serious cardiac effects is correlated with

A
  • Increasing dose of anthracycline
  • Increasing patient age
  • Presence of underlying heart disease.
221
Q

The incidence of congestive heart failure is dose related and ranges from approximately:

5% at ____mg/m2
Greater than 30% at ____ mg/m2

A

5% at 550 mg/m2
Greater than 30% at 600 mg/m2

222
Q

Diagnosis of Neutropenic Enterocolitis is confirmed by

A

Sonography or CT scanning

Mucosal thickening and polypoid appearance

223
Q

Management of Neutropenic Enterocolitis

A

Bowel rest, nasogastric suction, fluids, and antibiotics

  • Parenteral alimentation is sometimes used but is generally not helpful.
  • Right hemicolectomy is usually done only in the absence of resolution and if hemodynamic stability is lost
224
Q

Definition of Remission

A
  • Neutrophil count greater than 1 × 109/L
  • Platelet count greater than 100 × 109/L
  • Less than 5% blasts in the marrow by microscopy
  • Absence of extramedullary AML

Remission with incomplete platelet recovery CRplatelets (CRp) has all these requirements, but the platelet count does not reach 100 × 109/L.

225
Q

Early death can occur during induction chemotherapy, and ____________and ___________are the most important predictors of treatment-related mortality.

A

Performance status and age

226
Q

The two most compelling determinants of a poor outcome in AML

A

Older age and less-favorable cytogenetic risk

WILLIAM'S HEMATOLOGY (109 decks)

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