Protein Electrophoresis (PEL)

plasma protein distribution

60% albumin

35% globulin

4% fibrinogen

1% other

serum PEL

normal PEL graph

hypoalbuminemia

1. less production by liver / liver dz

2. inc loss or degradation

3. malnutrition

4. hormone therapy

5. pregnancy

alpha 1-antitrypsin (a1-AT)

• if a1-AT defic → liver (cirrhosis) & lung (emphysema) dz

• thyroid-binding globulin inc/dec

• alpha acid glycoprotein - ulcerative colitis

• malignancy & acute inflammation (acute-phase reactants) can inc the alpha1 protein band

alpha 2

ceruloplasmin - copper transport - dec in Wilson’s vs Menke syndrome (eye w golden ring)

alpha 2 macroglobulin - liver & renal dz → large size prevent loss from renal & inc production by liver

haptoglobin - absent in intravascular hemolysis

inc as APR

selective protein loss

long term loss of albumin & IgG in kidney

dec albumin, inc a2 & B globulins

nephrotic syndrome

beta 1

transferrin

inc in Fe defic

asialated transferrin - when transported into CSF leading to double transferrin peak

in microcytic anemia

due to iron defic - TF inc, ferritin

or chronic dz - no clear inc/dec

beta 2

fibrinogen: pseudoparaprotein, incomplete clot - heparinized pt (use EtOH to ppt out)

CRP: inflammation > 10, hs

CRP 2-3 = 3x risk for ACS

IgA

Gamma

acute/chronic inflammation

IgA/G/M or D/E ?

acute inflammation

inc a1 & a2 globulins

often w dec albumin

due to infn, injury, surg trauma

chronic inflammation

inc gamma globulin

inc a2 globulins

± a1 globulins

dec albumin

in autoimmune dz, chronic liver dz, chronic infn, malignancy

monoclonal gammopathy

alter B-cells produce an abnormal Ig paraprotein → assoc w benign monoclonal gammopathy & multiple myeloma

monoclonal gammopathies

most IgG > M > A > > light chain only »D/E

adverse properties of Ab

1.ability to agglutinate rbc

2. insolubility at low temp (cryoglobulinemia)

3. inc viscosity (Waldenstrom macroglobulinemia)

4. deposition in tissues w resulting organ dysfunction (eg AL (light chain) amyloidosis or Ig deposition dz

5. neuropathy

Beta-Gamma bridging

polyclonal inc in IgA extending into beta region

this pt also shows dec albumin

cirrhotic liver dz

malignancy

inflammatory dz

normal variant → do nothing

a1-antitrypsin

nephrotic syndrome??

beta gamma bridging - inc of IgA

also dec albumin

acute phase reactants

chronic inflammation

immunosuppressed / deficient

hypogammaglobulinemia

• congenital or

• acquired

  • multiple myeloma

  • primary amyloidosis

  • CLL

  • lymphoma

  • nephrotic syndrome

monoclonal gammopathy

immunofixation (IgM kappa)

permits detection & typing of monoclonal Ab or Ig in serum or urineim

immunotyping (capillary electrophoresis)

myeloma categories

MGUS < 3 g

smoldering > 3 g

MM > 3 g & clinical symptoms

multiple myeloma

CRAB

Calcium

Renal dz

Anemia

Bone lesions

urine PEP

help dx & monitor MM

may be ordered as f/up to otehr lab tests ie abnormal total protein & or albumin level

** urine PEP is more sensitive than serum PEP

usually ordered in difficult cases

serum vs urine PEP graph

CSF contains all proteins present in serum smaller quantities

char features

• prominent pre-albumin bnad

• double beta transferrin band

• most pt w multiple sclerosis have permanently observable oligoclonal bands - 2 or more bands in CSF & no bands in the serum

multiple sclerosis

sine qua non of MS is symptomatic ep are “separated in space & time” ie occur months/years apart ie weak arm → can’t control baldder

acute phase reactants

a1-antitrypsin → damages liver/lungs

chronic inflammation

Beta-Gamma bridging

immunodeficient or myeloma

multiple myeloma

myeloma