Plasma Cell Myeloma and Plasmacytoma

Question 1

Plasma Cell Myeloma and Plasmacytoma

What is the most common plasma cell neoplasm?

Answer 1

Multiple myeloma

Question 2

Plasma Cell Myeloma and Plasmacytoma

Multiple myeloma is more common in (males vs females)?

Answer 2

Males

Question 3

Plasma Cell Myeloma and Plasmacytoma

Little is known about the cause of multiple myeloma. However, according to reports, this environmental exposure has been associated with the development of the disease.

Answer 3

petroleum products

Question 4

Plasma Cell Myeloma and Plasmacytoma

All cases of myeloma are preceded by MGUS.

TRUE or FALSE?

Answer 4

True

Question 5

Plasma Cell Myeloma and Plasmacytoma

The bone disease that
arises in myeloma appears to be mediated in part by the inhibition of the RANK
pathway and the amplification of the Wnt signaling pathway.

TRUE or FALSE?

Answer 5

False.

other way around

Question 6

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Clinical finding in MGUS?

Answer 6

No organ damage

Question 7

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Clinical finding in smoldering multiple myeloma?

Answer 7

No myeloma-defining events or amyloidosis

Question 8

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Clinical finding in multiple myeloma?

Answer 8

One or more myeloma-defining events

Question 9

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Clinical finding in plasma cell leukemia

Answer 9

Organ damage
leukocytosis
high tumor burden
high proliferation rate

Question 10

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Status of Marrow disease/M-protein features

MGUS

Answer 10

<10% clonal plasma cells

Question 11

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Status of Marrow disease/M-protein features

smoldering multiple myeloma

Answer 11

10 to 60% clonal plasma cells

and/or

M-protein ≥30 g/L (or urinary M-protein ≥500 mg per 24 h)

Question 12

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Status of Marrow disease/M-protein features

multiple myeloma

Answer 12

> 10% clonal plasma cells or biopsy-proven plasmactyoma

Question 13

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:

Status of Marrow disease/M-protein features

plasma cell leukemia

Answer 13

Plasma cells in peripheral blood
≥2 x 10^9/L or ≥20%

Immature plasma cells in bone marrow

Question 14

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:
Management

MGUS

Answer 14

Monitor

Question 15

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:
Management

smoldering multiple myeloma

Answer 15

Close follow-up

Question 16

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:
Management

multiple myeloma

Answer 16

Chemotherapy

Question 17

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:
Management

Plasma cell leukemia

Answer 17

High-dose chemotherapy

Question 18

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:
Transformation rate

MGUS

Answer 18

0.5% to 1% per year

Question 19

Plasma Cell Myeloma and Plasmacytoma

Re: Spectrum of myeloma:
Management

smoldering multiple myeloma

Answer 19

10% per year (up to 20)

Question 20

Plasma Cell Myeloma and Plasmacytoma

Multiple Myeloma

When is the diagnosis made?
Criteria

Answer 20

> 10% clonal plasma cells (BM) or biopsy proven bone or extramedullary plasmactyoma

+
one or more myeloma-defining events

Question 21

Plasma Cell Myeloma and Plasmacytoma

Multiple Myeloma

What are the myeloma-defining events?

Answer 21

end-organ damage attributed to myeloma

CRAB

C-hypercalcemia (>2.75 mmol/L)

R-renal insufficiency (crea >177umol/L or CrCl <40mL/min)

A-anemia (<10 g/dL or >20 g/L below lower limit of normal)

B-bone lesions (one or more osteolytic lesions on x-rays, CT, or PET scan)

Question 22

Plasma Cell Myeloma and Plasmacytoma

Multiple Myeloma

In the absence of myeloma-defining events, what are the alternatives to diagnosis?

Answer 22

one or more biomarkers for malignancy
SLiM (sixty percent, light chains, MRI)

clonal bone marrow plasma ≥60%

involved:uninvolved light chain ratio ≥100,

1 focal lesions on MRI (≥5mm in size)

Question 23

Plasma Cell Myeloma and Plasmacytoma

Where is the most common location of solitary plasmacytoma?

Answer 23

Vertebra

Question 24

Plasma Cell Myeloma and Plasmacytoma

Standard initial imaging?

Answer 24

Skeletal survey

Question 25

Plasma Cell Myeloma and Plasmacytoma What is the historical staging system used for myeloma?

Answer 25

Durie Salmon Staging

Question 26

Plasma Cell Myeloma and Plasmacytoma Identify the criteria for historical stage 3 (using Durie Salmon)

Answer 26

Hemoglobin <8.5 g/dL Serum calcium >12 mg/dL Advanced lytic bone lesions High M component (IgG > 7 g/dL, IgA > 3 g/dL, and urine light chains >12 g/24 h)

Question 27

Plasma Cell Myeloma and Plasmacytoma Identify the criteria for the R-ISS Staging of Multiple Myeloma Stage I

Answer 27

Serum β2 microglobulin <3.5 mg/L and Serum albumin ≥35 g/L and standard risk chromosomal abnormalities by iFish and normal LDH

Question 28

Plasma Cell Myeloma and Plasmacytoma Identify the criteria for the R-ISS Staging of Multiple Myeloma Stage II

Answer 28

Neither R-ISS stage I nor III

Question 29

Plasma Cell Myeloma and Plasmacytoma Identify the criteria for the R-ISS Staging of Multiple Myeloma Stage III

Answer 29

Serum β2 microglobulin ≥5.5 mg/L and either high-risk chromosomal abnormality (by iFISH) "OR" high LDH

Question 30

Plasma Cell Myeloma and Plasmacytoma What is an important unfavorable factor with respect to local control after RT in SP?

Answer 30

tumor bulk Tumors <5 cm achieved a high level of local control with 35 Gy, whereas those ≥5 cm had a local failure rate of 58% (7 of 12 patients, total dose range 25 to 50 Gy).

Question 31

Plasma Cell Myeloma and Plasmacytoma An extramedullary presentation has been consistently demonstrated to have a significantly higher risk of subsequent development of myeloma with a 10-year rate of 76% compared with a bony presentation where the 10-year rate was 36% TRUE or FALSE?

Answer 31

false. bony > extramedullary

Question 32

Plasma Cell Myeloma and Plasmacytoma Where there was an elevation of M protein pretreatment, persistence of the M protein following radiation therapy (RT) predicts for progression to myeloma. TRUE or FALSE?

Answer 32

True

Question 33

Plasma Cell Myeloma and Plasmacytoma What are the cytogenetic abnormalities that are considered poor prognostic factors in myeloma as detected by FISH?

Answer 33

del(17p) t(14;16) t(4;14)

Question 34

Plasma Cell Myeloma and Plasmacytoma What is the standard treatment for SP?

Answer 34

RT

Question 35

Plasma Cell Myeloma and Plasmacytoma Is RT still indicated for a completely excised(GTR) SP of the spine?

Answer 35

Yes. RT is still indicated because of a high likelihood of microscopic residual disease.

Question 36

Plasma Cell Myeloma and Plasmacytoma In multiple myeloma, what is the preferred initial treatment for patients that are candidates for ASCT?

Answer 36

Bortezomib

Question 37

Plasma Cell Myeloma and Plasmacytoma Most common adverse reaction to Bortezomib?

Answer 37

neuropathy

Question 38

Plasma Cell Myeloma and Plasmacytoma Most common adverse reaction to Thalidomide?

Answer 38

DVT

Question 39

Plasma Cell Myeloma and Plasmacytoma Multiple Myeloma In patients not eligible for transplant, what chemotherapy (alklyating agent) is used in treatment in combination with bortezomib?

Answer 39

Melphalan+Prednisone (MP)

Question 40

Plasma Cell Myeloma and Plasmacytoma Multiple Myeloma In patients not eligible for transplant, what is the preferred treatment when an alkylating agent use is not applicable due to its toxicity?

Answer 40

lenalidomide+dexamethasone (LD) | FIRST trial

Question 41

Plasma Cell Myeloma and Plasmacytoma In multiple myeloma, what is the preferred combination treatment + conditioning for patients that are candidates for ASCT?

Answer 41

conditioning: melphalan chemo: RVD (lenalidomide, bortezomib + dexamethasone)

Question 42

Plasma Cell Myeloma and Plasmacytoma In multiple myeloma, what group of patients benefit the most in tandem transplantation?

Answer 42

those who did not achieve a 90% reduction of disease after the first ASCT.

Question 43

Plasma Cell Myeloma and Plasmacytoma In multiple myeloma, what is the maintenance therapy post ASCT?

Answer 43

1 y lenalidomide

Question 44

Plasma Cell Myeloma and Plasmacytoma In multiple myeloma, what is the most commonly used chemotherapy in relapse after ASCT (median 2 years)?

Answer 44

Bortezomib and Lenalidomide

Question 45

Plasma Cell Myeloma and Plasmacytoma Relapsed Multiple Myeloma What is a novel lMiD that has also demonstrated efficacy in relapsed myeloma, even in patients refractory both to bortezomib and lenalidomide?

Answer 45

Pomalidomide

Question 46

Plasma Cell Myeloma and Plasmacytoma What is the monoclonal antibody targeting a glycoprotein SLAMF-7 used in relapsed multiple myeloma?

Answer 46

Elotuzumab

Question 47

Plasma Cell Myeloma and Plasmacytoma What is the monoclonal antibody targeting CD38 used in relapsed multiple myeloma?

Answer 47

Daratumumab

Question 48

Plasma Cell Myeloma and Plasmacytoma TBI is now rarely used in multiple myeloma as a conditioning prior to ASCT due to toxicity concerns. However, for historical/theoretic purposes, what dose will you prescribe if the need arises?

Answer 48

8 Gy in 4 fractions A phase III French study (IFM [Intergroupe Francophone du Mye’lome] trial 9502) examined melphalan 200 mg/m2 alone (M200) versus melphalan 140 mg/m2 with TBI, (M140/TBI), and found that patients in the TBI-containing arm suffered more grade 3 or 4 mucosal toxicity, heavier transfusion requirement, and longer hospitalization stay. There was a higher toxic death rate in the M140/TBI arm (3.6% vs. 0% for the M200 arm).

Question 49

Plasma Cell Myeloma and Plasmacytoma What is the usual total dose for palliation of bone pain in multiple myeloma?

Answer 49

25 Gy When RT is given for pain because of disease involving a long bone, a local field suffices. It is unnecessary to treat the entire bone. Doses of 10 to 20 Gy (in 5 to 10 fractions) are effective, although the pain relief is often partial. Leigh et al. found a symptomatic response rate of 97% (complete pain relief in 26% and partial relief in 71%) after an average dose of 25 Gy given to 306 sites in 101 patients. There was no dose–response relationship above 10 Gy. Recurrence of symptoms requiring further treatment was seen in 6% of sites after a median of 16 months. Similar results are also reported by Matuschek et al., with complete pain relief in 31% and partial relief in 54%, with median RT dose of 25 Gy.

Question 50

Plasma Cell Myeloma and Plasmacytoma When using RT for SP, when do you include nodal irradiation if there are no gross nodes involved?

Answer 50

EMP (extramedullary) bulky disease close to draining lymph node station. For EMP, nodal involvement at presentation is observed in 10% to 20%, and occasional nodal failure in the literature led to a common practice of extending the RT coverage to the draining lymph node region.

Question 51

Plasma Cell Myeloma and Plasmacytoma What is the target for RT of bone SP?

Answer 51

gross tumor with margin. | not necessarily entire bone

Question 52

Plasma Cell Myeloma and Plasmacytoma Expansion to GTV for RT of SP?

Answer 52

Generally 1 to 3 cm 0.5 to 1 cm in the axial plaine 2-3 cm proximal and distal in long bones

Question 53

Plasma Cell Myeloma and Plasmacytoma SP What is the total RT dose range for small tumors? (<5 cm)

Answer 53

35 to 40 Gy

Question 54

Plasma Cell Myeloma and Plasmacytoma SP What is the total RT dose range for bulky tumors? (>5 cm)

Answer 54

45 to 50 Gy