Plasma Cell Dyscrasias

Question 1

MGUS

Answer 1

Precursor to Multiple Myeloma
No symptoms - 1% chance to develop myeloma
Lower protein level, lower abnormal level of plasma cells in marrow, no end organ damage
Criteria: M prot

Question 2

Multiple Myeloma

Answer 2

Ca of plasma cell. Overgrowth of plasma cell.
Lots of M protein
Men, african, elderly
IgA and IgG is most commonly elevated for heavy chain, light chain is kappa or lamda
Symptoms: bone pain/fractures, numbness in legs, wt loss, weakness, altered mental, SOB, fatigue
Think CRAB - Calcium high, Renal involve, Anemia, Back/Bone pain.
Criteria: Presence of serum M protein, Presence of clonal (all have same defect) plasma cells in bone marrow, Presence of end organ damage related to disease (from high calcium)
Durie salman staging
International Staging system
- stage 1 - B2M 5.5 mg/L
big diff in survival
Tx: smoldering - watch/wait
Chemotherapy
Steroids
Immunomodulators - very good
Bone marrow transx - not a curable cancer, so you get your own marrow, not someone elses - prolongs remission. auto transx
Bisphosphonates - for bone dz
Tx based on risk:
very rarely use allograft stem cell transplant. high morbidity. goal is prolongation in survival.
Single and double auto transplant -
immediate vs delayed transx?
Continue therapy until plateau phase.

Question 3

Waldenstroms Macroglobinemia

Answer 3

Plasma cell discrasia and need to have abnormal clonal lymphocytes in marrow
Macroglobulinemia: elev IgM
IgM in bone blood and Lymphoplasmactytic lymphoma in marrow
Related to IgM: neuropathy, coomb’s +, etc.
Dx: IgM monoclonal gammopathy, Bone marrow >10% monoclonal lymphocytes AND plasma cell differentiation
Tx like a lymphoma:
- asymptomatic - observe; Symptomatic - chemo;
Risk of progression is highest in first 5 years
Incurable - livable

Question 4

Plasmacytoma

Answer 4

Mass of plasma cells
Humerus>femur>pelvis>vertebrae
Low levels of M protein
Serum Ig levels preserved
Extramedullary: higher chance of successful tx. URT most common.
Dx: Biopsy, need clonal plasma cells, Normal skeletal imaging, no end organ damage, bone marrow with no evidence of plasma cells. low M prot
Tx: localized. Radiation tx, Extramedullary can be surgically removed. Rare
Bone: disappearance of M prot once tumor removed. 20% cured at 10 yr
Extramedullary: 70% cure at 10 yr. respond better to tx.

Question 5

Amyloidosis

Answer 5

Protein confirmation disorder
clonal plasma cells
extracellular deposition of light chain fragments in insoluble form. worry about what organs are deposited. cant get rid of one deposited
10% of myeloma pnts have amyloidosis
Screen:
MPEV: or SPEP, almost always have elevated light chains.
Dx: congo red stain iwth red green birefringence from biopsy

Question 6

Amyloidosis

Answer 6

Protein confirmation disorder
clonal plasma cells
extracellular deposition of light chain fragments in insoluble form. worry about what organs are deposited. cant get rid of one deposited
10% of myeloma pnts have amyloidosis
Screen:
MPEV: or SPEP, almost always have elevated light chains.
Dx: congo red stain iwth red green birefringence from biopsy
Poor prognostic factor: cardiac infiltration worst, autonomic neuropathy
Tx: no optimal tx, aim is to retard further deposition