Plasma Cell Neoplasms

Question 1

Myeloma: prognostic results:

Answer 1

• age
• PS
• beta2 microglobulin
• Albumin
• creatinine
• Ca
• LDH
• CRP
• BMAT % plasma cells
• cytogenetics/FISH
• gene expression profile

Question 2

Myeloma:adverse cytogenetic

Answer 2

T(4;14)
T(14;16)
T(14;20)
17p-
1p-
1q+

Question 3

Types of Monoclonal gammopathies

3

Answer 3

1. CAD
2. MGRS
3. MGUS (IgM and Non-IgM)

Question 4

Features of CAD

Answer 4

• AIHA
• Clonal B-cells
• IgM (usually Kappa)
• Binds RBCs (@ 0-4’C)
• I-Ag
• Activates compliment
• Extravascular haemolysis

Question 5

Definition of MGRS

Answer 5

1. Protein urea >1g/dL
2. Proggressive/subacute renal injury
3. Kidney biopsy: Injury 2’ to monoclonal Ig
4. No CRAB

Question 6

Definition of MGUS

Answer 6

1. Mprot < 30
2. BM PCs < 10%
3. No CRAB

Question 7

IgM MGUS Progression

Answer 7

Mayo Clinic RF:
* Mprot >15
* > SFLCR
* > Non IgG

I. IgM MGUS, PC type = MM
* t(11;14)
* Clonal PCs, No clonal Bs, MYD88 WT

II. IgM MGUS, NOS = LPL

Note:
* 1%/year = SMM
* 8-10yr = MM

Question 8

Definition of SMM

Answer 8

1. Mprot >30g/L
2. uMprotein > 500mg/24hr
3. BM PCs 10-60%
4. No CRAB

Question 9

Prognosis of SMM

Answer 9

Increased risk of progression
1. t(11;14)
2. del17p
3. 1q abberations

High Risk (20x3 rule)
1. Mprot > 20
2. SFLCR > 20/< 0.2
3. PCs >20%

Note:
10%/yr for 1st 5yrs = MM
3%/yr for next 5 years

Question 10

Definition of MM

Answer 10

1. Mprot >30g/L
2. uMprot > 500mg/24hr
3. BM PCs >60%
4. SlimCRAB
   * 60% PCs
   * LC Ratio (I:U) >100
   * MRI >1 focal lesions
   * Ca > (>2.75/>0.25 of ULoN)
   * Renal impariment (Creat >200/eGFR < 40)
   * Anaemia (HB < 10)
   * Bone disease (1+ lytic lesions)

Question 11

Prognosis of MM

Biochemical, Genetics and Morphology

Answer 11

I. Biochemical:
* B2-microglobulin
* LDH

II. Genetics:
* del17p
* TP53
* t(4;14), t(14;16), t(14;20)
* amplification of 1q
* del13
* Non hyperdiploid
* Double/Triple Hit

III. Morphology
* > vascularity of BMT
* PC Infiltrate:
* Extent (packed)
* Pattern (nodular)
* Cytological fts
* Anaplastic morphology

Note: Bartl & Frisch
1. High grade - PBs
2. Intermediate - dysplasia
3. Low grade - normal PCs

Question 12

Treatment approach in MM

Answer 12

I. Transplant eligible
II. Risk

I = Yes & II = Standard:
* VRD 3-4x
* HSCT
* Len Maintenance

I = Yes & II = High
* Dara + “
* HSCT
* Bort + “

I = No & II = Standard
* VRD 8-12x
* Len Maint

I = No & II = High
* VRD 8-12x
* Bort + “

Question 13

Regimens in MM Tx

Answer 13

Triplet/Quadruplet;
* AntiCD38 (Daratumumab/Isatuzumab)
* PI (Bortezomib, Carfilzamib, Ixazimib)
* IMID (Len, Thal, Pomalidomide)
* Dexa

Newer:
* BCMA
* CART
* AntiBCMA Mab
* BiTEs
* ADC