B3.043 Plasma Cell Myeloma Flashcards
what are plasma cell neoplasms?
neoplasms of terminally differentiated, Ig secreting plasma cells
plasma cell proliferation and monoclonal Ig (M-component)
who primarily develops plasma cell neoplasms?
middle aged and older adults
what are 3 types of plasma cell neoplasms
plasma cell myeloma (multiple myeloma)
monoclonal gammopathy of undetermined significance (MGUS)
primary amyloidosis
-lamba Ig chain folds and causes organ dysfunction
define plasma cell myeloma
multifocal destructive infiltration of plasma cells in bone marrow
define MGUS
M-component without features of myeloma
1% per year transformation into plasma cell myeloma
what are some clinical features of plasma cell myeloma`
CRAB criteria -bone pain, fractures, hypercalcemia -Renal disease -Anemia, thrombocytopenia -punched out Bone lesions infections
primary cause of death in people with plasma cell myeloma
infection or renal disease
what are some features of plasma cell myeloma in the blood
monoclonal immunoglobulin or light chain
-spike on serum protein electrophoresis
serum free light chains
Rouleaux formation (red cells line up in columns bc protein content is high)
describe the appearance of electrophoresis in clonal vs of variable specimens
clonal will separate into a well defined band
-10-20% of chains need to be clonal to make a defined band/spike on a graph
variable will appear to be a smear
plasma cell myeloma findings in urine
Bence Jones protein (monoclonal free light chain)
what is included in gamma on a serum protein electrophoresis
Igs, fribrinogen, c-reactive protein
what would an increased gamma signify if it was polyclonal
non-specific
chronic inflammation
liver disease
what would a decreased gamma signify
hypogammaglobulinemia
consider further evaluation for a paraprotein
what would a monoclonal band signify
M-protein (paraprotein)
what is immunofixation electrophoresis?
detection of M-protein
-higher sensitivity
determination of Ig class
-G,A,M,K,L, tested (doesn’t detect E or D paraproteins)
how are serum free light chains measured
measurement by nephelometry using an antibody to a region of the light chain normally not accessible in intact Ig molecules
high sens
why is the K/L ratio useful
can confirm monoclonality
determines light chain class
why are K and L levels useful
disease progression and monitoring
what is the function of 24 h urine studies
detection of monoclonal free light chains (MFLC) (Bence Jones protein) total protein urine protein electrophoresis -low sense urine immunofixation -high sense
what 3 things are required for plasma cell disorder diagnosis
- M-protein
- direct measurement from SPE
- serum free light chains
- urinary MFLC/BJP - bone marrow clonal plasma cells
- flow cytometry
- K/L immunohistochemistry - related organ or tissue impairment
- hypercalcemia
- renal insufficiency
- anemia
- bone lesions
describe plasma cell myeloma findings in the bone marrow
> 10% monoclonal plasma cells
nodules or diffuse sheets of plasma cells
CD38+
CD138+
clinical features of plasma cell myeloma: bone related
bone pain fractures hypercalcemia punched out bone lesions osteolytic factors from plasma cells - increased osteoclastic activity, decreased osteoblastic activity
clinical features of plasma cell myeloma: renal
cast nephropathy- light chain aggregates form tubular casts direct tubular toxicity amyloidosis (AL type) light chain deposition in interstitium hypercalcemia, hyperuricemia
clinical features of plasma cell myeloma: anemia, thrombocytopenia
bone marrow replacement by plasma cells
clinical features of plasma cell myeloma: infections
impaired humoral immunity
what is the best way to treat plasma cell myeloma
induction followed by hematopoietic stem cell transplant, if possible
drugs/therapies for multiple myeloma
immunomodulatory agents proteasome inhibitors steroids cytotoxic drugs (cyclophosphamide) monoclonal Ab (against CD 38) HDAC inhibitors
therapies for bone disease
bisphosphonates
radiation
therapies for hypercalcemia
hydration
bisphosphonates
therapies for hyperviscosity
plasmapheresis
therapies for anemia
transfusion
EPO
therapies for infection
vaccines
antimicrobial prophylaxis
