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25 Cards in this Set

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- multiple myeloma
- Lots and lots of plasma cells – hard to find normal precursors (not many neutrophils, or RBCs, why anemic)
a
- Monoclonal lambda staining pattern associated with multiple myeloma in BM
- in situ hybridization
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- Monoclonal lambda staining pattern associated with multiple myeloma in BM
- in situ hybridization
a
- Both the kappa and lambda in situ hybridization in BM studies show positive staining (blue cells)
- This is indicative of a polyclonal staining pattern
a
Rouleaux common in hyperviscoscity syndrome
a
- Light chain cast nephropathy - (Myeloma kidney)
- Way too much space b/t tubules and glomeruli b/c you have so many plasma cells
– not the actualy problem, its b/c Ig is despositing in the tubules
- giant cells often seen
a
- HandE stain of heart
- amyloid fibrosis
a
- AF
- congo red stain
a
- AF
- EM of fibrils
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Amyloidosis Involving glomerulus
a
Amyloidosis - Liver
a
Amyloidosis-Heart
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- Marrow is hypercellular. Waldenström's Macroglobulinemia - No fat is seen. Higher power view shows a mix of plasma cells and lymphocytes.
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- Marrow is hypercellular. Waldenström's Macroglobulinemia - No fat is seen. Higher power view shows a mix of plasma cells and lymphocytes.
a
Multiple myeloma - pathophys
- clonal, neoplastic prolif of plasma cells – mainly in BM - they live longer than they should so accumulation
- path happens after class switch
- need IL-6 to antagonize apop
Multiple myeloma - genetics
- aneuploid in up to 80%
- Hyperdiploidy most common
- abnormal karyotype in 50%
- chrom 1 and 13 have poor prognosis)
- 14 (heavy chain gene)
- cyclin D1 -> cell division
- c-myc -> gene rearrange
- inc Bcl-2
- if p53 mutant, chemo resistant
Multiple myeloma - Epi and Sx
- disease of old (avg 65)
- Weight loss or other B sxs (but much less pronounced)
- Sxs of anemia, and bone pain
- blacks twice as likely
Multiple myeloma - Lab stuff
- Rouleaux on the blood smears -> lots of circulating IG
- protein/albumin discordance
- Hypercalcemia from bone degradation
- Creatinine due to renal failure
- SPEP – lets us know if there’s a monoclonal protein (G id 60%, A is 20%)
Amyloidosis
- AL associated with MMy and abnormal light chains
- will stain + with Congo red stain by light microscopy (peach color)
- kidney and heart most likely to be involved
- dx w/ congo red, SC fat asp, renal or rectal biopsy
- treat like MP - can do transplant
Waldenström's Macroglobulinemia
- Disease of older adults
- low grade lymphoma involving BM and spleen
- Lymphoplasmacytic, mature B-cell, w/ monoclonal IgM
- sx are cold agglut, blurred vision and hypervis, can have amyloidosis
- Dx - BM, spleen showing IgM
- treat w/ plasmapherisis to remove IgM
- can do chemo w/ alks and steriods, analougs, or rituximab
Multiple Myeloma - major criteria
1) Plasmacytoma on tissue biopsy
2) BM plasmacytosis >30%
3) Monoclonal serum protein
Multiple Myeloma - minor criteria
- BM plasmacytosis 10-30% w/ a smaller monoclonal spike
- Lytic bony lesions
- Depressed normal Igs
Multiple Myeloma - treatment
- chemo immediately
- Melphan + prednisone (MP)
- VAD via IV if advanced
- Thalidomid if refractory - can combine w/ Dexamthasone
- Autologous stem cell transplant in younger
- Bortezomib (VELCADE) – proteosome inhibitors
- modulate w/ IFN-a
- maintain w/ steriods
Multiple Myeloma - adjuvant care
- Bisphosphonates - Pamidronate and Zoledronate - decrease Ca2+, prevent pathologic fractures –
- EPO
- G-CSFs raises neutrophils, may prevent infections and prophylactic may decrease infections too - both controversial
Monoclonal Gammopathy
- precursor for MMy - 1/4 progress
- serum M protein < 3 g/dL; < 5% marrow plasma cells
- no urine M, lytic lesions, anemia, hypercalcemia, or renal failure
- protein/albumin is normal!! ~2