Plasma cell dysplasia Flashcards
is polyclonal rise in immunoglobulins a normal reactive response or plasma dell dysplasia
normal reactive response
is monoclonal rise in immunoglobulins a normal reactive response or plasma dell dysplasia
plasma cell dysplasia
monoclonal rise in immunoglobulins as a result of plasma cell dysplasia results in
all antibodies being identical (monoclonal)
= paraprotein formation
does paraprotein formation come from monoclonality or polyclonality
monoclonality
what happens to the kidneys when there is increased immunoglobulin production
cant cope = renal impairment
excrete paraproteins as bence-jones proteins
bence jones proteins
myeloma
blood film appearance of RBCs coated in proteins (ie in plasma cell dysplasia)
roulaeux formation (RBCs staked like a set of coins)
differentials of plasma cell dysplasia (4)
myeloma
monoclonal gammopathy of undetermined significant (MGUS)
AL amyloidosis
waldenstroms macroglobulinaemia
investigations for plasma cell dysplasia
electrophoresis
serum immunofixation
blood film
quantify amount of paraproteins (myeloma high, MGUS low)
what does electrophoresis tell you in plasma cell dysplasia
if paraproteins are present
what does serum immunofixation tell you in plasma cell dysplasia
classify type of abnormal protein (which Ig)
what is myeloma
neoplastic disease of plasma cells
what happens to plasma cells in myeloma
clone of faulty plasma cells = increased antibodies (monoclonal antibodies) = paraprotein formation n
type of Ig most common in myeloma
IgG (can get any)
why does myeloma present with bone pain
increased oestoClasts = Chew up bone
what electrolyte imbalance occurs in myeloma and causes most of the symptoms
hypercalcaemia
presentation of myeloma (5)
bone kidney stones psychiatric moans abdo groans thirst/dehydration
bone pain
‘punched out’ lytic lesions
‘pepper pot’ skull
pathological fractures (from weakened bone)
myeloma
investigations for myeloma
electrophoresis
ESR
skeletal survey - to see which bones affected
ESR in myeloma
raised
chronic management of myeloma
chemo + steroids/bisphosphonates/thalidomide
symptomatic management of myeloma
analgesia (NOT NSAIDs bc renal impairment) for pain
bisphosphonates - for bone pain and hypercalcaemia
prognosis of myeloma
is it curable
not curable
aim for remission, will probs relapse
most common cause of paraproteinemia (presence of paraproteins)
monoclonal gammopathy of undetermined significance (MGUS)
what is monoclonal gammopathy of undetermined significance (MGUS)
when paraproteins are present but at v low concs = asymptomatic, doesn’t cause harm
who likely has asymptomatic monoclonal gammopathy of undetermined significance (MGUS)
old people! so don’t worry if you find it
does monoclonal gammopathy of undetermined significance (MGUS) lead to myeloma/waldenstoms
v v rarely
what happens to the paraproteins in AL amyloidosis
they clump together = amyloid formation = accumulation in tissues = serious organ damage
investigations for AL amyloidosis
congo red stain - ‘apple green bifringence’ under Polarised light
urinalysis - for proteinuria
EHCO - to check heart
LFTs - to check liver
congo red stain - ‘apple green bifringence’
amyloidosis
management of AL amyloidosis
how does it work
chemo
switches off light chain production = no more amyloid production = no further organ damage (can undo whats been done though)
what type of cancer is waldenstroms macroglobulinaemia
non hodgkins lymphoma
what type of Ig paraprotein is in waldenstroms
IgM
lol only thing you know but give yourself a pat on the back
what is significant about the IgM paraprotein in waldenstroms macroglobulinaemia
what does this cause
IgM is a tetramer = big protein = MACROglobulinaemia
cause hyperviscosity bc so big!
presentation of hyperviscosity in waldenstroms macroglobulinaemia
bleeding
fatigue
coma
confusion
presentation of waldenstroms macroglobulinaemia
like lymphoma! - night sweats, weight loss, fatigue
investigations for waldenstorms macroglobulinaemia
increased viscosity
protein electrophoresis - to see IgM paraproteins
management of waldenstroms macroglobulinaemia (3)
plasmapheresis - remove and replace patient plasma with non IgM rich plasma
chemo - long term
proteasome inhibitors - causes apoptosis of proteins
