5 - Plasma Cell Dyscrasias Flashcards
Multiple myeloma?
Malignancy of clonal plasma cells leading to monoclonal immunoglobulins
- spike of M protein/paraprotein
What do plasma cells make?
IgA IgG IgM IgE IgD
Multiple myeloma is characterized by?
Replacement of bone marrow
Bone destruction
What is MGUS?
Monoclonial gammopathy of undetermined significance
- milder version of multiple myeloma
- be careful with it though, it could go of for like no reason
MGUS is defined as?
<10% monoclonia plasma cells in bone marrow
Serum M-protein <3g/dL
No end organ damage
MGUS previlance?
1% of all adults
>70=3%
Approx 1% convert to multiple myeloma. Per year
Descrube a Multiple myeloma pt
Age 65
Male
Black:White 2:1
S/s of MM?
Related to bony destruction
Bone pain - back, hips, ribs Pathologic fractures Kidney disease Anemia Infection Spinal cord compression Hyperviscosity syndrome
What are hyperviscosity syndromes?
Mucosal bleeding Vertigo Nausea Visual disturbances Altered consciousness
MM PE?
Pallor Bone TTP/fx Soft tissue mass Neurologic signs - cord compression/neuropathy Fever - encapsulated organisms Renal failure
Why does MM cause renal failure/symptoms?
Hypercalcemia
Hyperuricemia
MM labs?
Anemia SPEP/UPEP w monoclonal spike M component w sharp peak in gamma/beta globulin region IgG (60%) IgA (25%) light chains Hypercalcemia - 25% Acute kidney injury
MM SPEP?
Pic of albumin and gamma spike
- looks like vampire teeth for kids
Slide 12
Rouleaux formation =?
MM
Pic on slide 13
Imaging for MM?
Skeletal survey (not bone scan) - lytic lesions
MRI or CT/PET
- extent of disease
MM diagnosis requires?
All 3 of:
- M-protein in serum/urine
- Bone marrow aspirate >10% plasma
- End-organ damage from plasma cells
- min 1 crab signs
(MM)
CRAB
CRAB
C - hyperCalcemia
R - Renal injury
A - anemia
B - lytic bone lesion
What will smoldering MM look like?
Same as MM except:
3: NO end-organ damage from plasma cells
- 2 signs of crab
MGUS requires what surveillance?
Q 3-6 mo
- SPEP/UPEP
- Hgb/Hct
- calcium
- renal panel
Bone marrow biopsy (1 x only)
Skeletal surveys (not regularly)
MM tx?
Chemo +/- allogeneic stem cell transplant
Localized radiation for bone pain
tx hypercalcemia and uric acid (aggressively)
Biphosphonates decreased fractures
Inpatient tx for MM-induced hypercalcemia?
- IVF
- furosemide (IV) (only if hypervolemic)
- biphosphonate (IV)
Resolves hypercalcemia in >90% of cases
MM survival?
Median survival of 5 yrs
What is waldenstrom macroglobulinema?
B cells that are morphologically a hybrid of lymphocytes and plasma cells
- produce abnormal IgM monoclonal protein
So fucked up B cells that make bad IgM
MM produces what kind of Ig?
Abnormal
IgG - 80%
IgA - 20%
Who gets waldenstrom macroglobulinemia?
60-70y/o
S/s of waldenstrom macroglobulinemia?
Fatigue Hyperviscosity Mucosal and GI bleeding AMS: lethargy, stupor, coma Cold agglutinin disease/peripheral neuropathy - 2/2 IgM paraprotein
Waldenstrom macroglobulinemia PE?
Hepatosplenomegaly Lymphadenopathy Engorged retinal veins Purpura NO bone TTP
Main Difference between MM and waldenstrom macroglobulinemia?
MM
- bone pain
- renal disease
WM
- NO bone pain
- renal disease uncommon
Labs for WM?
Anemia (rouleaux formation)
Renal disease is uncommon
WM diagnosis?
Monoclonial IgM spike on SPEP/UPEP
> /= 10% of bone marrow demonstrating infiltration by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation
WM tx?
plasmapheresis
- Hyperviscosity syndrome (tx and prophylaxis)
Rituximab
= anti CD20 B cell monoclonal antibody
Refractory WM tx?
Autologous stem cell transplant
- relapsed/refractory pts
Survival of WM?
Medial survival = 5yr
15 yr survival = 10%
