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Hematology Pro > Multiple Myeloma > Flashcards

Multiple Myeloma Flashcards

1
Q

What is the prognosis of Solitary Plasmacytoma (Of Bone / Of Extramedullary Tissues?)

A
  1. SBP:
    mOS of 10 years
    50-60% progress to MM
    ~10% local recurrence
  2. SEP:
    mOS of 12.5 years
    10-15% progress to MM
    7% local recurrence
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2
Q

Risk Factors for SBP / SEP progression to Multiple Myeloma:

A

SEP:

  • Minimal marrow involvement (20% risk of progression)
  • Location (H&N less risky than other soft tissue lesion)

SBP:

  • Clonal disease detectable at any limit
  • Older age [>60] (inc. relapse)
  • Axial skeletal involvement
  • Tumors <5cm
  • Immunoparesis
  • Osteopenia
  • Persistent M-protein >5g/L 5 years from diagnosis/treatment
  • Abnormal FLC ratio
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3
Q

Front-line treatment for AL Amyloidosis in:

a) Transplant eligible
b) Transplant ineligible

A

A) Dara - CyBorD or CyBorD

B) DaraCyBorD, CyBorD, BMT (Bortez, Mel, Dex), MD (Mel, Dex) [If notable neuropathy precluding bortez]

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4
Q

Treatment for Relapsed/Refractory AL Amyloidosis:

A

Single-agent Daratumumab
DRd, DVd, DPd
Ixazomib/Dex, Vd, Rd, Pd

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5
Q

t(11;14) is often linked to increased resistance to Bortezomib based regimens. What novel agent and its target does t(11;14) demonstrate sensitivity to?

A

A) Venetoclax, targeting BCL2

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6
Q

What are the criteria for HSCT in AL Amyloidosis?

A

Mayo Criteria:

  1. Physiological Age <70
  2. SBP >90 mmHg
  3. NYHA I-II, ECOG 0-2
  4. Troponin T <0.06
  5. CrCl > 30 (if above are met but patient on dialysis can consider)

Other considerations:

  1. No large pleural effusions or O2 dependent
  2. No more than 2 organs severely involved
  3. Factor X levels <25% (are associated with TRM of 50%)
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7
Q

What is the dose modification for Bortezomib based on peripheral neuropathy?

A

Grade 1 (asymptomatic, loss of deep tendon reflexes or paresthesia without pain or loss of function): No action required.

Grade 1 (with pain) or Grade 2 (interfering function but not activities of daily living): Reduce by one level (from 1.5 mg/m2 to 1.3 mg/m2; or from 1.3 mg/m2 to 1 mg/m2; or from 1 mg/m2 to 0.7 mg/m2).

Grade 2 (with pain) or Grade 3 (interfering with activities of daily living): Hold until resolution, may reinitiate at 0.7 mg/m2 once weekly.

Grade 4 (life-threatening, disabling, eg, paralysis): Discontinue.

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8
Q

How is AL Amyloidosis diagnosed?

A
  1. Presence of symptoms attributable to end-organ damage of amyloid deposition
  2. Prove the presence of amyloid deposition in an end-organ (typically BMBx + Fatpad biopsy. If negative then can consider either rectal biopsy for another random site, or organ-specific biopsy pending on risks)
  3. Prove the amyloid deposition is of light-chain type (Either electron-immunohistochemistry of mass spectrometry)
  4. Prove a clonal plasma cell disorder (M-protein in serum/urine, SFLC abnormality, or Clonal PCs)

ONLY can diagnose with ALL 4!

Otherwise you may misdiagnose for example ATTR-Amyloid with unrelated MGUS

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9
Q

What is POEMS syndrome stand for, and what is the diagnostic criteria?

A 
POEMS:
Polyneuropathy (100% of patients)
Organomegaly (50%)
Endocrinopathy (~66%)
Monoclonal protein (100% of patients)
Skin Changes (~66%)

Mandatory: Polyneuropathy and Monoclonal Protein (Almost always lambda, but not necessary for dx)

Major:

  1. Elevated VEGF (~66%)
  2. Castleman’s disease (15-25%)
  3. Osteosclerotic Bone Lesions (97% of patients)

Minor:

  1. Organomegaly
  2. Extravascular volume overload
  3. Skin changes
  4. Endocrinopathy
  5. Papilledema
  6. Thrombocytosis or Polycythemia

Diagnosis: Both mandatory, and 1 major, 1 minor

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10
Q

How is POEMS Syndrome treated?

A

There is no RCT evidence to guide treatment. In general:

Localized (1-3 bone lesions): Radiotherapy

Systemic: A MM based treatment regimen, with consideration for Melphalan-AutoHSCT

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11
Q

How do you stage Multiple Myeloma? (ISS, R-ISS)

A

ISS:
I: Albumin >35, B2M <3.5
II: Not I or III
III: B2M >5.5

R-ISS:
5yr OS (I: 82%, II: 62%, III: 40%)
I: ISS I and LDH normal and no high-risk cytogenetics
II: Not R-ISS I or III
III: ISS III AND (EITHER High-risk cytogenetics OR High LDH)

R-ISS High-risk cytogenetics:
t(4;14), t(14;16), del(17p)

NOT in R-ISS but also bad:
Gain (1q), Amplification (1q), del(12p), t(8;14) - MYC-IgH

Other common mutations:
t(11;14), t(6;14), Trisomies/Hyperdiploidy

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12
Q

What is belantamab mafodotin? What is it’s specific toxicity?

A

Antibody drug conjugate used in MM
Anti-BCMA linked to aurostatin immunotoxin
ORR= 60% in heavily pre-treated pts, PFS 14 months.

Has specific toxicity of corneal toxicity and secondary visual problems. Common toxicity of myelosuppression and infusion reactions.

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13
Q

Name common myeloma translocations and their partners?

A

t(4;14) - MMSET/FGFR3 + IGH
t(4;16) - c-MAF + IGH
t(8;14) - MYC + IGH
t(14;20) - MAFB + IGH

t(11;14) - CCND1 + IGH
t(6;14) - IRF4 + IGH

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14
Q

What non-malignant complications occur from IgM antibodies?

A
  1. Cryglobulinemia
  2. Cold Agglutinin Disease
  3. Schnitzler Syndrome (autoimmune with fever, bone/joint pain, chronic hives)
  4. CANOMAD Chronic ataxic neuropathy, ophthalmoplegia, IgM-MGUS, cold agglutinin, and disialosyl antibodies
  5. Sensorimotor neuropathy (against myelin-associated protein: ANTI-MAG)
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15
Q

Explain the difference between stringent CR vs CR in MM

A

CR:

  1. Undetectable M-protein in Serum/Urine by Immunofixation
  2. BM PC <5%
  3. No plasmacytomas

sCR:

  1. CR plus
  2. Normal FLC
  3. No clonal plasma cells on BMBx by either IHC or Flow cytometry
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16
Q

What is VGPR in MM?

A

VGPR:

  1. Serum and urine M-protein detectable by immunofixation but not on electrophoresis OR
  2. > 90% reduction in serum M-protein AND urine M-protein level < 100 mg/24 h
17
Q
  1. What is a CR in AL Amyloidosis?

2. What is VGPR in AL Amyloidosis

A
  1. Negative SPEP/UPEP on IFE AND Normal FLC

2. dFLC of 40mg/L

18
Q

What is PR or PD in MM?

A

PR:
> 50% reduction of serum M-protein and reduction in 24 hours urinary M-protein by >90% or to < 200 mg/24 h

If the serum and urine M-protein are unmeasurable, a > 50% decrease in the difference between involved and uninvolved FLC levels is required in place of the M-protein criteria

If serum and urine M-protein are not measurable, and serum free light assay is also not measureable, > 50% reduction in plasma cells is required in place of M-protein, provided baseline bone marrow plasma cell percentage was > 30%

In addition to the above listed criteria, if present at baseline, a > 50% reduction in the size of soft tissue plasmacytomas is also required

PD:
Increase of > 25% from lowest response value in any one or more of the following:
Serum M-component and/or (the absolute increase must be > 0.5 g/dL)
Urine M-component and/or (the absolute increase must be > 200 mg/24 h)
Only in patients without measurable serum and urine M-protein levels; dFLC level (The absolute increase must be > 100 mg/L)
Bone marrow plasma cell percentage; the absolute percentage must be > 10%
Definite development of new bone lesions or soft tissue plasmacytomas or definite increase in the size of existing bone lesions or soft tissue plasmacytomas
Development of hypercalcaemia (corrected serum calcium > 11.5 mg/dL or 2.65 mmol/L) that can be attributed solely to the plasma cell proliferative disorder

19
Q

What is PR or PD in AL Amyloidosis?

A

PR:
Requires any of the following:
≥ 50% reduction in serum monoclonal protein levels (if > 0.5 g / dL or > 5 g / L at baseline)
≥ 50% reduction in urine light chains (if >100 mg / day at baseline)
≥ 50% decrease in difference between the involved and uninvolved serum free light chain (if >10 mg / dL or >100 mg / L at baseline)

PD:
Requires any of the following:
If progressing from CR, any detectable monoclonal protein or abnormal free light chain ratio (light chain must double)
If progressive from PR or SD, ≥ 50% increase in the serum M protein to > 0.5 g/dL, or ≥ 50% increase in urine M protein to > 200mg/day with visible peak present.
Serum free light chain increase of ≥ 50% to > 10 mg/dL (100 mg/L)

NB: PD in MM is only 25% increase, rather than 50% in Amyloidosis (and most measurement systems)

20
Q

A: What is the Mayo Model for risk of progression from SMM to MM?
B: What are other risk factors for progression of SMM to MM

A

A:
20/20/20 Rule:

Serum M-protein >20mg/L
BM PCs >20%
Ratio of involved:uninvolved light chains >20

Low-risk group (0) - 10% at 2 years, 5% per year for 10 years
Intermediate-risk group (1) - 30% at two years, 7 percent per year for the next three years, and 4 percent per year for the next five years.
High-risk group (>2)- 48% at two years, 11 percent per year for the next three years, and 3 percent per year for the next five years

B:
BM PCs >95% clonal
Immunoparesis

21
Q

Side-effects of Lenalidomide:

A 
Cytopenias (especially Neutropenia and thrombocytopenia)
VTE (Requires ASA prophylaxis)
Infection
Diarrhea (Can be late-onset)
Rash
Secondary malignancies
Muscle cramps
Affects ability to be able to mobilize stem cells for auto-SCT
22
Q

Smoldering multiple myeloma definition:

A

-Serum monoclonal protein (IgG or IgA) ≥30 g/L OR
urinary monoclonal protein ≥500 mg per 24 h and/OR
clonal bone marrow plasma cells 10% to 60%

-Absence of myeloma-defining events or amyloidosis

23
Q

What are the adverse effects of Bortezomib (+PI based regimens)

A

Peripheral neuropathy, most common with Bortezomib
GI Side-effects (Constipation possible, Diarrhea possible)
Rash
VZV reactivation (all patients require proph)
Cardiac toxicity (mostly with Carfilzomib, but all PIs)
Thrombocytopenia
Not renal dependent (But Carfilzomib is and can cause renal tox during infusion)

24
Q

Bisphosphonate/Denosumab in Myeloma; Cautions and Benefits

A

Benefits:
Reduce number of vertebral fractures
Reduce skeletal related events (Compound outcome of hypercalcemia, patho # and lytic lesions)
Reduces associated bony pain
Generally no improvement of PFS/OS (Maybe Z.A. improves this, but only 1 trial demonstrated that)

Cautions:
ONJ -> Risk is dental work. With-hold 1 month prior to extractions, and only resume once healing complete
Hypocalcemia
Atypical #

25
Q

How do you stage MGUS (I.e. Mayo risk score)

A

M protein ≥15 g/L
Non-IgG MGUS (ie, IgA, IgM, IgD MGUS)
Abnormal SFLC ( <0.26 or >1.65)

The absolute risk of disease progression over 20 years for patients with various combinations of risk factors is:

3 risk factors (high-risk MGUS) — 58% (~3%/yr)
2 risk factors (high-intermediate risk MGUS) — 37% (~2%/yr)
1 risk factor (low-intermediate risk MGUS) — 21% (~1%/yr)
no risk factors (low-risk MGUS) — 5% (~0.25%/yr)

26
Q

What is TEMPI Syndrome?

A 
Telangectasias
Erythrocytosis with elevated EPO
MGUS
Perinephric fluid collections
Intrapulmonary Shunting

Responds to treatment with MM-like therapy. Presumptive relation to Plasma cell dyscrasia based off this observation.

27
Q
  1. What are risk factors for poor stem cell collection for autologous donation?
  2. What can be given to improve this?
A 
Older age
Marrow-damaging chemotherapy (alkylators, purine analogues)
Lenalidomide
Radiation to marrow
Uncontrolled marrow disease

Plerixafor can improve this.

28
Q

What are risk factors for osteonecrosis of the jaw with anti-resorptive therapy

A

Type of bisphosphonate (z.a. > pamidronate)
Longer duration of therapy
Poor dentition
Dental procedures

Hematology Pro (5 decks)

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