Plasma Cell Neoplasm Pathology Flashcards
Multiple Myeloma is a clonal neoplastic proliferation of ______.
mature plasma cells
Each clone of MM produces a single Ig, which are typically
- IgG
- IgA
- Bence-Jones proteins (free light chains)
_____ (cytokines) → increases plasma cell proliferation
IL-6
How does renal insufficiency develop in MM?
Bence-jones proteins are renally excreted → proteinuria/light chain toxicity
(some light chains also predispose to amyloidosis)
Russell bodies (cytoplasmic) or Dutcher bodies (nuclear)
(PAS+)
… are found in which 2 neoplasms?
- MM
- Waldenstrom
Mott cell
cell w/many Russel Bodies
(found in MM; note clock-faced nucleus)
Flame cells are due to _____ at the periphery
Ig
(found in MM)
MM flow cytometry markers (3)
CD38+, CD138+, CD19+
(contrast w/B cell CD20+, CD19+)
MM is due to a _____ (2) deletion or ____ duplication or _____ translocation.
- 13q or 17p → LOF p53
- 1q
- t(4;14)(p16;q32)
Genetic pathway affected by MM
MAPK → RAS → c-myc activation (the event that transforms it into MM)
MM clinical presentation (6)
- anemia (normocytic, normochromic)
- bone pain + fatigue
- Elevated Creatinine
- hematologic malignancy sx: fever, wt. loss, fatigue
- peripheral neuropathy
- renal insufficiency
_____ (lab finding) that confirms MM.
M-spike: dense band on electrophoresis → shine a light through it and it gives you this graph
(albumin is the band to the left; Igs are in the gamma band)
Once you find the M-spike, what is the next step?
immunofixation to detect the isotype
(IgG & lambda present in the example)
Diagnostic criteria for MM includes > 10% clonal plasma, CRAB criteria. What is the CRAB mn?
(the opposite of MGUS which has <10% & NO CRAB sx)
C: increased calcium
R: renal insufficiency
A: Anemia
B: bone pain (osteoporotic)
Most sensitive imaging for “punched out lesions” of MM
MRI
Staging of MM is based on _____(4).
- kidney function
- beta-2 microglobulin
- LDH
- cytogenetics
MC cause of death in MM
- infection
- kidney failure
prognosis of MM
50% at 5 years
Clinical course of MM w/non-BMT tx
Monoclonal gammopathy of Undetermined Significance (MGUS) is a production of low-levels of _____.
Igs
(clonal plasma cells in BM; plasma cell dyscrasia)
MGUS affects 1 in _____ people of 50 years old
100! (mostly asymptomatic)
(african americans and white males)
Right: could be due to inflammation
________ has >10% BM cells and NO light chain restriction.
Reactive Plasmacytosis (aka polyclonal plasmacytosis)
25% of ______ transforms into MM
Reactive Plasmacytosis (aka polyclonal plasmacytosis)
(tx: observation only)
Smoldering myeloma is the middle ground between ______ (2 diseases)
MGUS & MM
(no CRAB manifestations)
elevated plasma cells, elevated IgM, and an asymptomatic presentation is likely ______
smoldering myeloma
(tx: observation only)
Monoclonal IgM (M-protein), malignant lymphoplasmacytic cell infiltration of BM, hepatosplenomegaly and lymphadenopathy, hyperviscosity indicate ________ (dx)
Waldenstrom Macroglobulinemia (aka lymphoplasmacytic lymphoma)
Waldenstrom Macroglobulinemia (aka Lymphoplasmacytic Lymphoma) is a _____ - grade lymphoma.
low
Waldenstrom Macroglobulinemia (aka lymphoplasmacytic lymphoma) risk factors (3)
- Hep C (viral)
- Autoimmune (i.e. Sjogren’s)
- White Elderly Caucasian
MD88 activation on gene at 3p22.2 loci, CD20+, CD38+, CD138(-) → ______
Waldenstrom Macroglobulinemia (aka lymphoplasmacytic lymphoma)
(translocations rare)
______ is a distinguishing laboratory finding of Waldenstrom and likely the reason it remains asymptomatic
elevated serum Igs
(hypergammaglobulinemia)
Sequelae of Waldenstrom Macroglobulinemia (4)
- DLBCL (Richter)
- amyloidosis
- stroke
- cold agglutinin diz
- CHF
Tx for waldenstrome
- observe
- chemo
- BMT
Amyloidosis
accumulation of EC tissue deposition of fibrils (low molecular weight proteins (Ig light chains) in tissue
Amyloidosis may lead to ______ (4).
- nephrotic syndrome
- heart failure
- hepatomegaly
- neuropathy
(<10% of MM is a/w amyloidosis)
