Plasma Cell Disorders and Myeloma Flashcards
what makes antibodies
plasma cells
b cells
proteins are made up of how many heavy and light chains
2
2
which antibody is a dimer
IgA
which antibodies are monomers?
IgD
IgE
IgG
what decides what kind of antibody an Ig is going to be?
depends on the recombination of the VDJ region
once a B cell leaves a lymph node after fighting infection, what can it become?
a memory B cell in ciruclation OR
go back to the marrow as a plasma cell
what do plasma cells do
make antibodies
“clock face nucleus” on H and E stain
plasma cell
what colour is a plasma cell’s cytoplasm and why?
blue as its laden with protein
what is a paraprotein?
a monoclonical immunoglobulin
paraproteins are markers of…
underlying clonal B cell disorder
how is bence jones protein detected?
urine electrophoresis
what is bence jones protein?
immunoglobulin light chains
what is the relevance of BJP?
high BJP indicates an increase in light chains which = more monoclonal antibodies (myeloma) or more plasma cells
free light chain production by plasma cells is __g per day
0.5
what conditions can cause a paraproteinaemia?
myeloma
amyloidosis
lymphoma
if myeloma is asymptomatic is it malignant?
yes it can be
what 2 aspects of myeloma cause symptoms?
direct tumour cell effects
paraprotein mediated effects
symptoms of myeloma that result from the tumour cell?
bone lesions
inc calcium
bone pain
marrow failure
paraprotein symptoms of myeloma
renal failure
immune suppression
hyperviscosity
amyloid deposition
most common antibody produced in myeloma
IgG
“punched out, lytic lesions in the skull”…
myeloma
how do light chaisn cause renal damage?
light chains will be filtered through the glomeruli and form casts in the PCT
what kind of fractures are common in myeloma
wedge compression vertebral fractures
what effect do myeloma cells have on osteoblasts and clasts?
decreased osteoblasts
inc osteoclasts
symptoms of hypercalcaemia
bones stones moans groans thirst dehydration renal impairment
Tx cast nephropathy in myeloma
hydration
stop nephrotoxic drugs
steroids/chemo to switch off light chain production
median age of diagnosis of myeloma
65
Tx myeloma
steroids eg dexamethasone/prednisolone
cyclophosphamide
thalidomide/lenalidomide
high dose chemo/stem cell transplant in fit patients
how is myeloma monitored?
paraprotein levels
myeloma tends to relapse T or F
T
pre-cancer form of myeloma?
MGUS
symptom control of myeloma?
opiate analgesia
radiotherapy
bisphosphonates for bones
vertebroplasty
paraprotein level is
30
how to tell if its MGUS or myeloma?
paraprotein <30g/l
bone marrow plasma cells <10%
asymptomatic
Ix myeloma
BJP immunofixation calcium renal function Hb
how is the presence of a paraprotein confirmed?
immunofixation of serum and urine
pathophysiology of AL amyloidosis?
mutation in the light chain that causes amyloid to accumulate in the tissues
Tx AL amyloidosis
chemo
AL amyloidosis is a form of familial amyloidosis T or F
F different conditions
symptoms of AL amyloidosis
nephrotic syndrome
cardiomyopathy
organomegaly
malabsorption
Ix AL amylodosis
urinalysis
organ biopsy with congo red stain
SAP scan to look for other organ involvement
what is waldenstrom’s macroglobulinaemia?
neoplasm of cells that are an intermediate between lymphocytes and plasma cells
presence of IgM paraprotein….
waldenstroms macroglobulinaemia
signs of waldenstroms
lymphadenopathy splenomegaly marrow failure hyperviscosity neuropathy
symptoms of waldenstroms
fatigue visual disturbance confusion coma bleeding heart failure b symptoms eg night sweats, w loss
Tx waldenstroms
chemo
plasmaphoresis - removing IgM
