proteins and paraproteinemia Flashcards
what is the difference between serum and plasma?
in serum fibrinogen is removed
in plasma fibrinogen is still present
what are the major contributors to serum protein ?
albumin and immunoglobulins
what are the factors that increase serum protein ?
haemconcentration
increased synthesis
what are the factors that decrease serum protein ?
haemdilution
renal loss
gut loss
redistribution
what does serum electrophoresis separate ?
separates protein based upon size and charge
what must be used in electrophoresis ?/
serum not plasma
how many bands does serum separate into ?
normal serum separates into five bands
what is the most abundant plasma protein ?
albumin
what does the first main band in serum protein electrophoresis show?
Albumin
what does the second , third, fourth and fifth band in electrophoresis show ?
alpha 1 globulin
alpha 2 globulin
beta globulin
gamma globulin
what does the alpha 1 globulin band consist of ?
alpha 1 antitrypsin
when are the levels of alpha 1 antitrypsin increased and decreased?
increased in:
acute phase reactions
decreased in:
inherited deficiency
what happens to albumin levels in acute phase reactions ?
decreased levels
what does the alpha 2 globulin band consist of ?
alpha 2 macroglobulin
haptoglobulins
lipoproteins
when are the alpha 2 globulins increased?
acute phase reactions
nephrotic syndrome
chronic infections
what are the function of haptoglobulins ?
they bind free haemoglobin
when are haptoglobulins levels affected ?
increased in acute phase reactions
decreased in intravascular haemolysis
what is the structure of immunoglobulins ?
consist of a light chain and a heavy chain
immunoglobulins monoclonal or polyclonal ?
polyclonal
what is the cause of hypergammaglobulinaemia ?
chronic major infection
chronic liver disease
autoimmune disease
sarcoidosis
high igA is characteristic of ?
liver disease
what is paraproteinemia ?
the presence of monoclonal immunoglobulin band in the serum
what does a plasma cell look like histologically ?
eccentric nucleus
cartwheel appearance
surrounded by a lighter area
what is the main cause of M-Protein ?
MGUS
Multiple Myeloma
AL amyloidosis
what is a myeloma ?
cancer of plasma cells
what is the clinical presentation of multiple myeloma ?
hypercalcaemia renal failure anemia bone pain and pathological fractures hyper viscosity
what are the screening test done for multiple myeloma ?
full blood count ESR plasma viscosity urea creatinine and calcium serum immunoglobulins total protein and albumin concentrated urine for Bence jones proteins
what are bence jones proteins
immunoglobulin light chain that is found in urine
when are monoclonal free light chains found ?
found in serum when GFR is compromised
likely to be missed if serum is only tested urine must also be tested
what are the diagnostic investigations for multiple myeloma?
1.first paraprotein must be quantified🍗 immunofixation of serum and urine serum free light chains 2.bone marrow infiltration by neoplastic plasma cells☠️ bone marrow aspirate bone marrow trephine 3.demonstrate lytic bone lesions skeletal survey
after diagnosing multiple myeloma what is important to identify next ?
the type of immunoglobulin ( IgA, IgG…)
what is immunofixation ?
immunofixation always for the detection and typing of monoclonal antibodies or immunoglobulins in serum or urine ( what type of IG)
what may be seen in peripheral blood smear in multiple myeloma ?
rouleaux formation
what are the laboratory findings in multiple myeloma ?
high total protein paraprotein bence-jones proteins in urine renal impairment hypercalcaemia anemia ( normochromic normocytic)
when are proteins in the CSF elevated ?
with infection
contamination from blood
chronic inflammatory disorders
tumors
if the CT scan used to detect subarachnoid haemorrhages is equivocal what is the next step in management ?
look for CSF xanthochromia
what does xanthochromia mean ?
presence of bilirubin in the CSF
when should the CSF sample for xanthochromia be taken ?
take a sample a minimum of 12 hours post suspected bleed to allow for released haemoglobin to be converted into haemoglobin
when are oligoclonal bands found in the CSF ?
in inflammatory CNS disorders such as in MS