EXAM #3: PLASMA CELL DYSCRASIAS Flashcards
What is Multiple Multiple Myeloma?
Hyperproliferation of plasma cells
What is the abnormal protein that is produced in Multiple Myeloma?
M-protein i.e. elevation of monoclonal protein
What are the most common types of Multiple Myeloma?
1) IgG
2) IgA
3) Light chain only
What are the two types of light chains?
Kappa or Lambda
What is the difference between MGUS and Myeloma?
MGUS is “pre-cancerous”
What are the symptoms of Multiple Myeloma?
1) Lytic lesions on BONE SURVEY i.e. x-ray
2) Increased risk of fracture
3) Paresthesias
“Punched out” lesions
What are the criteria for diagnosis of Multiple Myeloma?
- Elevated serum monocloncal protein
- Abnormal plasma cells in bone marrow
- End organ damage
What are the indications of end organ damage?
1) Increased Ca++
2) Lytic bone lesions
3) Anemia
4) Renal failure
What is smoldering myeloma? How does this differ from MGUS?
- Smoldering is asymptomatic Multiple Myeloma without end organ damage and a monoclonal antibody concentration greater than 3 g/dL
- MGUS increased M-protein but less than 3 g/dL
What lab values are used for determining the stage of Multiple Myeloma?
- Serum albumin
- B2M
What is the general trend for serum albumin and B2M for staging of Multiple Myeloma?
Worse prognosis for:
- Decreasing albumin
- Increasing B2M
What chromosome is commonly mutated in Multiple Myeloma?
Chromosome 14
What is the “treatment” for smoldering Multiple Myeloma?
Watch and wait
- SPEP every 3-6 months
- Annual bone survey
Generally, how is Multiple Myeloma treated?
Chemotherapy Steroids Immunomodulators BM transplant Bisphosphonates/ Rad.
What is the difference between autologous and allogenic stem cell transplant?
Auto= from oneself* Allo= from matched donor
**This is what is more commonly done now
What is the goal of BM transplant?
Prolongation of survival
What drugs are used as maintenance therapy after auto stem cell transplant?
Thalidomide
Lenalidomide
What is a Plasmacytoma?
Single plasma cell tumor in the bone
What lab value is characteristic of Plasmacytoma?
Low or no levels of M-protein in the serum or urine
What type of Plasmacytoma has a better prognosis?
Extramedullary
How is Plasmacytoma diagnosed?
1) Biposy proven lesion of bone or soft tissue
2) Normal bone survey
3) No end organ damage
4) Bone marrow with no evidence of plasma cells
What is the treatment for Plasmacytoma?
- Radiation
- Surgery
What is the prognosis of Plasmacytoma?
Poor, most progress to Multiple Myeloma in 2 years
What is Amyloidosis?
- Amyloid = misfolded protein in the extracellular space
- Primary amyloidosis is the deposition of AL amyloid, which is derived from Ig light chain
How is Amyloidosis diagnosed?
1) SPEP
2) Urine free light chains
3) Serum free light chain
What is the definitive diagnosis for Amyloidosis?
Congo Red Stain with red-green birefringence on tissue biopsy
What are the poor prognostic factors of Amyloidosis?
1) Cardiac*
2) ANS neuropathy
3) Liver involvement w/ hyperbilirubinemia
4) Lack of suppression of underlying clonal disease
What is the current treatment for Amyloidosis?
Prevention of further amyloid deposition
What is Waldenstrom’s Macroglobulinemia?
Excess of IgM with lymphoma
What is the key symptom of Waldenstrom’s Macroglobulinemia?
Hyperviscosity Syndrome
How is Waldenstrom’s Macroglobulinemia diagnosed?
1) IgM monoclonal gammopathy
2) Lymphocyte involvement?
What is the treatment for Waldenstrom’s Macroglobulinemia?
Chemotherapy and plasmapharesis for symptomatic Waldenstrom’s Macroglobulinemia
