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WILLIAM'S HEMATOLOGY > 106 Myeloma > Flashcards

106 Myeloma Flashcards

1
Q

Represents the second most common hematologic cancer

A

Myeloma

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2
Q

MGUS is an asymptomatic condition that typically develops years before the diagnosis of myeloma and evolves to a lymphocytic or plasma cell neoplasm at a rate of approximately ______ per year

A

1% per year

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3
Q

Lifestyle and occupational factors associated with myeloma

A

High body mass index
Exposure to pesticides, organic solvents (benzene, petroleum derivatives, styrene), or chronic radiation
Thorium dioxide (thorotrast)
Exposure to acute radiation
Exposure to fresh wood, wood dust, or working in sawmill factories
Autoimmune diseases (especially rheumatoid arthritis and pernicious anemia) and chronic viral infections (such as HIV and hepatitis C)

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4
Q

Mutations more frequent in myeloma

A

Chromosome 13 deletions
RAS mutations
Non–Ig locus–associated MYC translocations

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5
Q

At early stages during the so-called _______________ phase, myeloma cells are totally dependent on marrow microenvironment to survive and on interleukin (IL)-6 and other cytokines.

A

Intramedullary phase

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6
Q

Most common genomic aberrations in MGUS

A

Hyperdiploidy

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7
Q

Most common genomic aberrations in Myeloma

A

Cyclin D dysregulation

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8
Q

Most common genomic aberrations in Plasma Cell leukemia

A

Del(13q)/monosomy13

PTEN losses, methylations of p14 promoter, and RB1 inactivations

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9
Q

Mutations that are present at similar rates in MGUS and myeloma.

A

Hyperdiploidy and IGH translocations (t(11;14), t(4;14) and MAF translocations)

**These abnormalities are not considered driver events in myeloma.

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10
Q

Traditionally, myeloma patients have been divided into 2 subgroups:

A

Hyperdiploid: more than 46 but less than 76 chromosomes (34–60% of myeloma)
Nonhyperdiploid: up to 44-45 chromosomes

**Pseudodiploid: 44/45 or 46/47 chromosomes with gains or losses
**Near-tetraploid

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11
Q

Characteristics of hyperdiploid patients

A

IgG kappa type with bone involvement
Gains of odd-numbered chromosomes, including trisomies of chromosomes 15, 9, 5, 19, 3, 11, 7, and 21 (ordered by decreasing frequency)
Favorable prognosis

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12
Q

Characteristics of hyperdiploid patients

A

IgG kappa type with bone involvement
Gains of odd-numbered chromosomes, including trisomies of chromosomes 15, 9, 5, 19, 3, 11, 7, and 21 (ordered by decreasing frequency)
Favorable prognosis

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13
Q

Employed to detect five major primary IGH translocations in myeloma, which occur more frequently in nonhyperdiploid patients (85% vs <30%).

A

Fluorescence in situ hybridization (FISH) analysis

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14
Q

Most frequent translocation (20% of cases)

A

t(11;14)(q13;q32)

Leading to upregulation of cyclin D1

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15
Q

Subgroup of cyclins with high level expression of cyclin D1, typically responds well to treatment, has an increased frequency of early relapse, but enjoys excellent long-term survival

A

CD-1 subgroup

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16
Q

Subgroup of cyclins with igh levels of cyclin D3, typically exhibits lymphoplasmacytoid morphology, and has a worse progression-free survival (PFS) and overall survival (OS)

A

CD-2 subgroup

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17
Q

MAF translocations include:

A

t(14;16) : c-MAF overexpression
t(14;20): deregulates MAFB
t(8;16): involving MAFA

**rare (5%, 2%, <1% of cases, respectively), but associated with poor prognosis

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18
Q

The prevalence of these three primary IGH rearrangements is similar in MGUS, indicating the need for additional transforming events to precipitate active myeloma.

A

t(6;14)
t(11;14)
t(14;16)]

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19
Q

Copy Number Alterations in Myeloma

Genomic aberrations associated with poor prognosis in myeloma patients

A

Deletion of chromosome 13
Deletion of chromosome 17p13
Amplification of chromosome 1q21

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20
Q

Deletion of chromosome ______________ affects 50% to 60% of newly diagnosed myeloma, is more frequent in the nonhyperdiploid group (>70%) in comparison to the hyperdiploid group, and often cooccurs with t(4;14) or t(14;16) translocations.

A

chromosome 13

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21
Q

Deletions of chromosome _______________are rare in newly diagnosed myeloma (5–10%), more common in relapsed and refractory cases (20–40%), and inevitably associated with a negative prognosis, causing early relapse in patients treated with or without autologous stem cell transplantation (SCT).

A

chromosome 17p involving the TP53 locus

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22
Q

Amplification of________ is detected by FISH in approximately 40% of newly diagnosed myeloma and in 70% of relapsed myeloma, and negatively affects OS, with a cumulative effect based on the number of 1q21 locus copies.

A

Amplification of 1q21

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23
Q

Translocation described with the TP53 genomic locus, causing simultaneous gain of 1q21 and deletion of 17p.

A

Jumping translocation of 1q12 (JT1q12)

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24
Q

5 of the 11 genes commonly mutated in myeloma

A

KRAS, NRAS, FAM46C, DIS3, and TP53

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25
Q

A transmembrane heparan sulfate bearing proteoglycan expressed during the plasma cell stage of B-cell maturation and can bind to type I collagen, thereby inducing expression of metalloproteinases and promoting bone resorption and invasion

A

CD138

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26
Q

Regulates not only specific homing of myeloma cells to the marrow but also mobilization or marrow egress, being possibly accountable for the multifocal marrow localization and blood circulation of myeloma cells

A

CXCL12 (also called SDF-1α)/CXCR4 axis

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27
Q

Binds to the RANK (receptor activator of NF-κB) receptor to stimulate osteoclast differentiation, formation, and survival; myeloma cells produce RANKL and upregulate RANKL expression in BMSCs and osteoblasts via direct contact interaction, signaling induction or production of IL-7.

A

Ligand for receptor activator of NF-κB (RANKL

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28
Q

A decoy receptor that normally prevents RANK–RANKL interaction via soluble factors, integrin α4β1- vascular cell-adhesion molecule (VCAM) 1 interaction, production of Dickkopf-1 (DKK1), or inactivation by syndecan-mediated internalization into myeloma cells.

A

Osteoprotegerin (OPG)

OPG levels are decreased in the serum of myeloma patients and correlate with lytic bone lesion development; a high RANKL-to-OPG ratio is associated with worse prognosis

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29
Q

TRUE OR FALSE

In a noninferiority, phase 3 double-blind study, denosumab was not inferior to zoledronic acid (Zometa), leading to its approval by the FDA in 2018.

A

TRUE

In a noninferiority, phase 3 double-blind study, denosumab was not inferior to zoledronic acid (Zometa), leading to its approval by the FDA in 2018.

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30
Q

TRUE OR FALSE

Bisphosphonates not only block osteoclasts and modulate osteoblasts but also have an effect on tumor burden.

A

TRUE

Bisphosphonates not only block osteoclasts and modulate osteoblasts but also have an effect on tumor burden.

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31
Q

Stimulate osteoclastogenesis and osteoclast activity

A

Receptor activator of nuclear factor-κB (RANK) receptor/receptor activator of nuclear factor-κB ligand (RANKL) a
Macrophage inflammatory protein (MIP)-1α

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32
Q

An inhibitor of osteoblast activity.

A

Dickkopf-1 (DKK1)

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33
Q

“CRAB” criteria

A

Hypercalcemia: serum calcium ≥11.5 mg/100 mL or
Renal insufficiency: serum creatinine >2 mmol/L)
Anemia: normochromic, normocytic with a hemoglobin
value of >2 g/100 mL below the lower limit of normal or a
hemoglobin value <10 g/100 mL
Bone lesions: lytic lesions, severe osteopenia, or pathologic
fractures

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33
Q

“CRAB” criteria

A

Hypercalcemia: serum calcium ≥11.5 mg/100 mL or
Renal insufficiency: serum creatinine >2 mmol/L)
Anemia: normochromic, normocytic with a hemoglobin
value of >2 g/100 mL below the lower limit of normal or a
hemoglobin value <10 g/100 mL
Bone lesions: lytic lesions, severe osteopenia, or pathologic
fractures

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34
Q

Myeloma defining biomarkers:

A

Clonal marrow plasma cell percentage equal to or greater than 60%
Ratio of involved versus uninvolved FLCs equal to or exceeding 100
Presence of more than 1 focal lesion on magnetic resonance imaging (MRI)

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35
Q

Criteria for Diagnosis of Myeloma

A

All 3 criteria must be met except as noted:
* Clonal marrow plasma cells ≥10% and/or presence of a biopsy-proven plasmocytoma
* Presence of serum and/or urinary monoclonal protein (except in patients with true nonsecretory myeloma)
* Evidence of end-organ damage

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36
Q

Most common symptom and laboratory feature

A

Monoclonal Ig peak on immunofixation of serum or urine (97%)

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37
Q

In contrast to other lymphoproliferative disorders, thrombocytopenia is uncommon at diagnosis, even with extensive marrow infiltration, possibly because _________has thrombopoietic activity

A

IL-6

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38
Q

TRUE OR FALSE

Bortezomib causes a cyclic thrombocytopenia with different kinetics from cytotoxic drugs. It appears during the first 10 days of each cycle, has a short recovery time, no cumulative or persistent effects, and there is an absence of marrow megakaryocyte toxic damage, as the thrombocytopenia primarily results from a functional alteration in platelet budding.

A

TRUE

Bortezomib causes a cyclic thrombocytopenia with different kinetics from cytotoxic drugs. It appears during the first 10 days of each cycle, has a short recovery time, no cumulative or persistent effects, and there is an absence of marrow megakaryocyte toxic damage, as the thrombocytopenia primarily results from a functional alteration in platelet budding.

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39
Q

Overt bleeding is a relatively uncommon presenting symptom for myeloma patients; however, it occurs more commonly with ___________

A

IgA paraproteins

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40
Q

Easy bruising is more common in _______________ (15–41% of patients at diagnosis)

A

Systemic AL amyloidosis

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41
Q

Drugs that have antiangiogenic properties and are associated with an increased risk of venous thromboembolism (VTE), ranging from 5% to 18% of treated patients, when combined with other chemotherapeutic agents or erythropoietic agents

A

(IMiDs), such as thalidomide, lenalidomide, and pomalidomide

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42
Q

The VTE prophylaxis of choice for patients who are at low risk for VTE

A

Aspirin

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43
Q

The M protein presents as a single narrow peak, migrating in the___________region of the densitometer tracing.

A

γ, or, less often, β

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44
Q

Test that identifies the unique and specific Ig idiotypes.

A

Immunofixation analysis

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45
Q

Most common M protein

A

IgG 60%

IgA 20%
Ig light chains alone 20%
IgD, IgM, and biclonal 5%

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46
Q

A low M-spike concentration is particularly suggestive of _____myeloma isotype

A

IgD myeloma isotype

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47
Q

Light-chain myeloma patients should be followed by _____________________; these patients present more often with renal failure or increased creatinine levels.

A

UPEP and urine immunofixation

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48
Q

Light-chain proteinuria is frequent, especially in _______ myeloma

A

IgD myeloma

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49
Q

TRUE OR FALSE

The κ light chain isotype is twice as common as the λ isotype

A

TRUE

The κ light chain isotype is twice as common as the λ isotype

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50
Q

The κ light chain isotype is twice as common as the λ isotype, except in _____ myeloma.

A

IgD myeloma

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51
Q

A technique used to detect monoclonal FLCs and provides an FLC κ:λ ratio

A

FLC assay (FREELITE assay)

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52
Q

The κ:λ ratio is considered abnormal if :

A

less than 0.26 (λ-restricted Ig light chain)
OR
more than 1.65 (κ-restricted Ig light chain)

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53
Q

Half-life of FLCs

A

2–4 hours

**Ig : 17–21 days

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54
Q

TRUE OR FALSE

SPEP can be used to detect early treatment responses and should be evaluated routinely in patients with AL amyloidosis and oligosecretory myeloma.

A

FALSE

FLC assay can be used to detect early treatment responses and should be evaluated routinely in patients with AL amyloidosis and oligosecretory myeloma.

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55
Q

TRUE OR FALSE

High baseline FLC correlates with shorter survival in newly diagnosed myeloma patients despite achievement of complete response (CR).

A

TRUE

High baseline FLC correlates with shorter survival in newly diagnosed myeloma patients despite achievement of complete response (CR).

***A rapid reduction in FLCs after therapy is also linked to inferior OS and event-free survival (EFS), suggesting the presence of highly proliferative myeloma cells, particularly sensitive to combination chemotherapy.

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56
Q

Plasma cells description

A

Abundant basophilic cytoplasm and round, eccentrically located nuclei, with “clock-face” or “spoke-wheel” chromatin without nucleoli; paranuclear clear area, deeply blue cytoplasm

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57
Q

Inclusion bodies in plasma cells

A

Russell bodies (cherry-red refractive round bodies)
Multiple pale bluish-white, grape-like accumulations (Mott-cells or Morula cells)
Crystalline rods
Glycogen-rich IgA (flame cells)

These abnormal cells are characteristic of plasmablastic myeloma, a poor prognostic type of myeloma with a high number of mitotic figures

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58
Q

TRUE OR FALSE

Myeloma cells are clonal by definition and produce both κ or λ light chains, which are present in the membrane surface but not on the cytoplasm

A

FALSE

Myeloma cells are clonal by definition and produce either κ or λ light chains, which are present in the cytoplasm but not on the membrane surface.

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59
Q

Immunophenotype of myeloma cells

A

CD138+, CD45−, CD38+, and CD19−, and are CD56+

A few cases are CD20+ or CD117+ (KIT), but responses to treatment with rituximab or imatinib mesylate are uncommon.

60
Q

If amyloid deposition is suspected, ___________staining can be performed on the marrow biopsy, showing diffuse involvement or focal perivascular niche localization of amyloid protein.

A

Congo red

61
Q

Microvessel density can be assessed by staining for endothelial markers such as

A

CD131 and CD34

62
Q

Corresponds to the percentage of plasma cells in the S phase of the cell cycle and has traditionally been measured by assessing the uptake of DNA precursors (tritiated thymidine or 5-bromo-2′-deoxyuridine [broxuridine]) during short in vitro culture periods.

A

Plasma cell labeling index

63
Q

Renal insufficiency is related to 2 major causes:

A

Myeloma cast nephropathy (also called light-chain cast nephropathy or myeloma kidney)

Hypercalcemia

64
Q

TRUE OR FALSE

THE urine dipstick can detect Bence Jones proteinuria, and it is a technique that should be used to assess the presence of a urinary M spike.

A

FALSE

The urine dipstick does not detect Bence Jones proteinuria, and it is therefore not a technique that should be used to assess the presence of a urinary M spike.

65
Q

TRUE OR FALSE

Lambda light chains tend to be more nephrotoxic than the κ type, and renal impairment can be present with modest λ light chain secretion.

A

TRUE

Lambda light chains tend to be more nephrotoxic than the κ type, and renal impairment can be present with modest λ light chain secretion.

66
Q

Management of hypercalcemia

A

Loop diuretic to promote calciuresis
Calcitonin
Slow infusion of a single dose of a bisphosphonate or denosumab

67
Q

A complex syndrome related to deposition in target organs, such as heart or kidney, of amyloidogeneic Ig light chains organized in fibrillary structures.

A

AL amyloidosis

68
Q

AL amyloidosis with renal dysfunction is more common in patients with λ light chains, especially those with the __________subgroup.

A

λ VI light-chain subgroup

69
Q

A safer alternative for bone-protective therapy in patients with advanced renal disease, as it is not renally excreted

A

Denosumab

70
Q

TRUE OR FALSE

Renal dysfunction is a negative prognostic factor, and it results in use of suboptimal therapies, longer hospitalization, and an increased risk of infection.

A

TRUE

Renal dysfunction is a negative prognostic factor, and it results in use of suboptimal therapies, longer hospitalization, and an increased risk of infection.

Hence, patients who recover normal kidney function have a better outcome compared to those who do not.

71
Q

Leading cause of morbidity and mortality in myeloma

A

Infections

72
Q

Responsible for a myeloma patient’s susceptibility to encapsulated organisms, such as Streptococcus pneumoniae and Haemophilus influenza

A

Hypogammaglobulinemia

73
Q

The invariant chain of the major histocompatibility class 1 molecule, which is shed by myeloma cells

Levels correlate with tumor burden and are important for patient staging using the International Staging System (ISS)

A

β2M

74
Q

Polyneuropathy, typically demyelinating, is a mandatory diagnostic criteria together with presence of a plasma cell disorder, generally λ-restricted

A

POEMS syndrome, or osteosclerotic myeloma

75
Q

Incidence of hyperviscosity syndrome

A

Myeloma: less than 10%
Waldenstrom macroglobulinemia: 30%

76
Q

Plasma cell leukemia is diagnosed when circulating plasma cells comprise more than _______% of white cells in the blood or exceed an absolute level of ______ x 109/L.

A

20% of white cells in the blood

Absolute level of 2x 109/L

77
Q

The presence of a clonal plasmacytic infiltrate outside the marrow

A

EXTRAMEDULLARY DISEASE

EMD only if the infiltrate is present at anatomic sites distant from the bone or adjacent soft tissue, hence excluding cases where soft-tissue masses arise in contiguity with the marrow

78
Q

Characteristics of EMD cells

A

CD56− or CD56low
Present t(4;14) and del(17p) more commonly
TP53 mutations, TP53 nuclear localization, or high expression levels of focal adhesion kinase

79
Q

TRUE OR FALSE

The use of chemotherapy concomitantly with radiotherapy is discouraged, as it may result in profound cytopenia

A

TRUE

The use of chemotherapy concomitantly with radiotherapy is discouraged, as it may result in profound cytopenia

80
Q

Treatment options for spinal cord compression

A

Local radiotherapy and/or decompressive surgery

Local radiotherapy using less than 30 Gy over the course of 5–6 weeks is potentially curative in the presence of a solitary plasmacytoma

Higher intensity radiotherapy protocol over the course of 5 fractions together with pulse steroids bortezomib may be used in patients with single site of EMD

81
Q

Treatment of choice if a singular vertebral collapse is evident without a plasmacytoma

A

Decompressive laminectomy

82
Q

Diagnostic procedure In patients with suspected signs/symptoms of AL amyloidosis such as heart failure, nephrotic syndrome, periorbital purpura or macroglossia

A

Fat pad aspirate or fat punch biopsy

83
Q

The ISS is based on two widely available parameters

A

serum β2M and albumin

84
Q

Stages of Myeloma based on ISS

A

Stage I: β2M <3.5 ALB ≥3.5

Stage II: β2M <3.5 ALB <3.5 OR β2M 3.5–5.5

Stage III:β2M >5.5

85
Q

Correlates with tumor mass and impairment in renal function

A

β2M

86
Q

Reflects the effect of IL-6 produced by the microenvironment of myeloma cells on the liver

A

Low albumin

87
Q

A complete skeletal survey includes:

A

Posteroanterior view of the chest; anteroposterior and lateral views of the cervical spine, thoracic spine, lumbar spine, humeri, femora, and skull; as well as an anteroposterior view of the pelvis

88
Q

Roentgenographically detectable osteolytic lesions require at least ________% loss of bone mass and hence represent advanced bone destruction.

A

50% to 70%

89
Q

Has been shown to provide statistically significant superior sensitivity compared with radiography for the detection of osteolytic lesions in patients with myeloma

A

Whole-body CT, either alone or in combination with PET

90
Q

The first-choice imaging technique to identify and to assess the extent of osteolytic lesions.

A

Whole-body CT

91
Q

Widely used in both newly diagnosed and relapsed myeloma and in the event of suspected cord compression

Has the ability to discriminate myeloma from normal marrow.

A

MRI

92
Q

Refers to a planned second course of high-dose chemotherapy followed by HSCT within 6 months of the first.

A

Tandem Stem Cell Transplantations

93
Q

A study that compared the addition of daratumumab (Darzalex), an anti-CD38 monoclonal antibody, to bortezomib, thalidomide, and dexamethasone (D-VTd) versus bortezomib, thalidomide, and dexamethasone (VTd) alone

A

CASSIOPEIA study

94
Q

TRUE OR FALSE

For newly diagnosed transplantation-eligible patients, combinations that include alkylating agents should be avoided as damage to normal hematopoietic stem cells can be incurred, which may render it impossible to collect stem cells for auto-HSCT. Lenalidomide may also hamper the collection of stem cells

A

TRUE

For newly diagnosed transplantation-eligible patients, combinations that include alkylating agents should be avoided as damage to normal hematopoietic stem cells can be incurred, which may render it impossible to collect stem cells for auto-HSCT. Lenalidomide may also hamper the collection of stem cells

95
Q

The traditional age limit for auto-HSCT has been ____ years,

A

65 years

96
Q

TRUE OR FALSE

Chronologic rather than physiologic age is more suitable for determining transplantation eligibility

A

FALSE

Physiologic rather than chronologic age is more suitable for determining transplantation eligibility

97
Q

A significant concern with maintenance therapy with__________ is the risk of secondary malignancy

A

Lenalidomide

98
Q

Options for maintenance therapy

A

Lenalidomide
Bortezomib
Ixazomib (Ninlaro)

99
Q

Study that evaluated ixazomib (Ninlaro) as a maintenance therapy following auto-HSCT

A

TOURMALINE-MM3 study

100
Q

Two phase II studies, that demonstrated activity of bortezomib in patients with relapsed or refractory myeloma.

A

SUMMIT and CREST

101
Q

Study which compared bortezomib with dexamethasone alone, found a median OS of 30 months in the bortezomib group versus 24 months in the dexamethasone group.

A

APEX study

102
Q

Granted accelerated FDA approval as a single agent for the treatment of patients who have received at least two prior lines of therapy

A

Carfilzomib

103
Q

IMiDs that are FDA approved for the treatment of relapsed or refractory myeloma

A

Thalidomide, lenalidomide, and pomalidomide

104
Q

Side effects of Thalidomide

A

Sedation, peripheral neuropathy, and severe constipation

105
Q

The most common side effects with Pomalidomide

A

Myelosuppression and infections.

106
Q

A phase 3 study comparing pomalidomide, bortezomib, and dexamethasone with bortezomib and dexamethasone in patients with prior lenalidomide exposure.

A

OPTIMISMM trial

107
Q

A potent cereblon E3 ligase modulator with immunomodulating and proapoptotic activity

A

Iberdomide (CC-220)

108
Q

A phase III, randomized trial, showed that the combination of vorinostat (Zolinza) and bortezomib was active and well tolerated.

A

Vantage 088 trial

109
Q

Common side effect of histone deacetylase inhibitors

A

Myelosuppression and diarrhea

110
Q

A monoclonal anti-CD38 antibody, was granted Fast Track Designation and Breakthrough Therapy Designation by the FDA based on results of a phase I/II trial that demonstrated single-agent activity in relapsed, refractory myeloma.

A

Daratumumab

** Isatuximab

111
Q

The bortezomib, dexamethasone, with or without daratumumab trial, favored the use of the triplet combination compared to bortezomib and dexamethasone alone.

A

Castor trial

112
Q

A study that compared lenalidomide and dexamethasone alone with lenalidomide, dexamethasone, and daratumumab.

A

POLLUX study

113
Q

A phase III randomized study, which compared IPd with pomalidomide and dexamethasone (Pd) for relapsed disease.

A

ICARUS

114
Q

A humanized monoclonal IgG1 antibody directed against human CS1 (also known as CD2 subset 1, SLAMF7, CRACC, and CD319), a cell-surface antigen glycoprotein that is highly expressed on myeloma cells and normal plasma cells, also has been approved by the FDA for the treatment of relapsed disease.

A

Elotuzumab (Empliciti)

115
Q

A a selective inhibitor of nuclear export.

It binds to__________ forcing the nuclear localization and functional activation of tumor-suppressor proteins, trapping inhibitor of nuclear factor κBα in the nucleus to suppress NF-κB activity, and preventing oncoprotein mRNA translation.

A

Selinexor (Xpovio)

Exportin 1 (XPO1)

116
Q

An afucosylated humanized IgG1 that binds specifically to BCMA and is conjugated to the microtubuledisrupting agent monomethyl auristatin-F (MMAF)

A

Belantamab mafodotin (GSK2857916)

117
Q

The most notable toxicity of Belantamab mafodotin

A

Keratopathy

118
Q

A selective oral inhibitor of B-cell lymphoma-2 (BCL-2)

Approximately, 20% of myeloma patients will exhibit t(11;14) associated with high BCL-2 expression

A

Venetoclax

119
Q

Bisphosphonates

A

Pamidronate (Aredia) and zoledronic acid

120
Q

RANKL inhibitors

A

Denosumab

121
Q

The IMWG and the National Comprehensive Cancer Network (NCCN) panels advocate the use of either a bisphosphonate or denosumab______________ for patients with myeloma and lytic bone disease

A

Monthly

122
Q

Doses of:
Pamidronate:
Zoledronic acid:
Denosumab:

A

Pamidronate: 90 mg delivered IV for at least 4 hours every 3–4 weeks
Zoledronic acid: 4 mg IV for 15 minutes every 3–4 weeks
Denosumab: 120 mg SQ every 4 weeks

123
Q

A key concern with bisphosphonates, especially zoledronic acid, is the risk of ________________

A

Osteonecrosis of the jaw (ONJ)

124
Q

Percutaneous procedures for treating compression fractures and also have been used in the setting of myeloma

A

Vertebroplasty (injection of methyl methacrylate or bone cement)

Kyphoplasty (use of an inflatable balloon followed by instillation of bone cement)

125
Q

The primary indication for the use of radiation therapy

A

Palliation of bone pain

Other indications include impending fracture, cord compression, or relief of symptoms associated with a mass (ie, cranial nerve palsies, cosmesis, or organ or joint dysfunction).

126
Q

TRUE OR FALSE

Most VTEs occur within the first 60 days of therapy, coinciding with maximum cytoreduction.

A

TRUE

Most VTEs occur within the first 60 days of therapy, coinciding with maximum cytoreduction.

127
Q

Three risk factors for lenalidomide-associated VTE

A

Higher dexamethasone doses, administration of erythropoietin (Epogen, Procrit), and concomitant administration of other agents

128
Q

TRUE OR FALSE

Therapy-related risk factors weigh heaviest in the determination of risk for VTE.

A

TRUE

Therapy-related risk factors weigh heaviest in the determination of risk for VTE.

129
Q

The following thromboprophylaxis is recommended:

A

(a) acetylsalicylic acid (aspirin) in either a standard dose of 325 mg/day or in a low dose of 81 mg/day for patients with one or no risk factor or

(b) low-molecular-weight heparin (LMWH) once a day, or full-dose warfarin for patients if two or more risk factors or therapy-related risks are present

130
Q

The recommended duration of prophylaxis

A

6–12 months.

131
Q

A ______% dose reduction should be made for bortezomib-induced grade 2 neuropathy while grade 3 or 4 requires _______________

A

50% dose reduction

drug discontinuation

132
Q

Preferred route of administration of Bortezomib

A

SQ

133
Q

Symptomatic treatment for thalidomide-induced and bortezomib-induced neuropathy

A

Gabapentin (Neurontin), pregabalin (Lyrica), or tricyclic antidepressants

134
Q

A severe “bone” disease, associated with bisphosphonate therapy that affects the jaws and typically presents as infection with necrotic bone in the mandible or maxilla

Presence of exposed bone in the maxillofacial region that does not heal within 8 weeks.

A

Osteonecrosis of the Jaw

135
Q

TRUE OR FALSE

Stable disease is no longer used as a measure of treatment efficacy, but rather time to progression, which is measured from the start of therapy and, importantly, includes all patients entered into clinical studies.

A

TRUE

Stable disease is no longer used as a measure of treatment efficacy, but rather time to progression, which is measured from the start of therapy and, importantly, includes all patients entered into clinical studies.

136
Q

TRUE OR FALSE

Dynamic changes in skeletal events readily identified by modern imaging techniques such as MRI and PET-CT are included in response assessments.

A

FALSE

Dynamic changes in skeletal events readily identified by modern imaging techniques such as MRI and PET-CT are excluded from response assessments.

137
Q

Disease monitoring should be performed at least every _______ for the first year and at a minimum _______________ thereafter.

A

every month

every other month

138
Q

The prognosis of myeloma is determined by 3 factors:

A

(a) host
(b) tumor biology and disease burden
(c) type of therapy applied

139
Q

Collections of monoclonal plasma cells originating either in bone or in soft tissue

A

Plasmacytoma

bone (solitary osseous plasmacytoma [SOP])

soft tissue (extramedullary plasmacytoma [EMP])

140
Q

Plasmacytomas comprise less than _____% of plasma cell neoplasms.

A

10%

141
Q

TRUE OR FALSE

Although patients with SOP and EMP can both progress to myeloma, persons with SOP progress in the majority of cases, in contrast to EMP, where only up to 50% eventually develop myeloma.

A

TRUE

Although patients with SOP and EMP can both progress to myeloma, persons with SOP progress in the majority of cases, in contrast to EMP, where only up to 50% eventually develop myeloma.

142
Q

Clinical features of congestive heart failure, nephrotic syndrome, malabsorption, coagulopathy, rash (oral mucosal rash, “raccoon’s eyes”), or neuropathy

A

Primary amyloidosis

143
Q

The primary difference between AL amyloidosis and LCDD

A

The difference in structure of the deposited protein; in AL amyloidosis, it is fibrillar versus granular in LCDD.

LCDD is usually associated with the κ light chain subtype, whereas AL amyloidosis is associated with the λ light chain subtype of myeloma

144
Q

The diagnosis of AL amyloid often can be made by

A

Fine-needle aspiration of SQ fat or by biopsy of the rectal mucosa

Although biopsy of accessible clinically involved tissue is preferable

145
Q

Staining the tissue with __________may reveal perivascular amyloid with its classical apple-green birefringence when viewed under polarized light.

A

Congo red

Thioflavin T is also a useful stain, producing intense yellow-green fluorescence in AL amyloidosis.

146
Q

Patients with SM should be followed at close intervals of every _________months

A

3–6 months

147
Q

Smoldering myeloma patients who may benefit from therapy based on IMWG

A

Patients who have greater than 60% marrow plasma cells, κ:λ FLC ratios greater than 100, and two lesions on MRI or PET-CT imaging

WILLIAM'S HEMATOLOGY (89 decks)

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