What lab value may be increased postprandially in people that are Lewis positive group B or O secretors?
Alkaline phosphatase (intestinal)
Medications (OCPs, NSAIDs) may elevate Alk Phos levels
Which liver disease:
1) is associated with marked polyclonal increase in IgG?
2) is associated with marked polyclonal increase in IgM?
1) Autoimmune hepatitis
2) Primary biliary cirrhosis
What are reasons for decreased albumin on SPEP?
Malnourishment (used to assess nutritional status)
Inflammatory states (negative acute phase reactant)
Increased catabolism: inc T4, pregnancy or steroids
Loss: nephrosis, burn, protein losing enteropathy
Fluid retention (SIADH)
What are reasons for increased or decreased alpha 1 band on SPEP?
Increased: Acute inflammatory states (acute phase reactant)
Decreased: alpha 1 anti-trypsin deficiency, HDL def and aged serum
1) What are the reasons for increased alpha 2 band on SPEP?
2) What two other proteins are represented in the alpha 2 band?
1) Relative concentration elevated in liver and renal disease; large size prevents its loss in nephrotic syndrome leading to a relative 10 fold rise in concentration
2) Ceruloplasmin (decreased in Wilson's disease, acute phase reactant) and Haptoglobin (binds free hemoglobin, acute phase reactant)
*NOTE low ceruloplasmin is not detectable with SPEP
What major protein is represented in the beta 1 region and what would cause this spike to increase?
It is markedly increased in iron deficiency
What major proteins are represented in the beta 2 region?
IgA and C3
*Fibrinogen, usually absent from serum, may be present in the beta-gamma interface when there is incomplete clotting (a possible pseudo M spike)
What major proteins are represented in the gamma region (gamma 1 and gamma 2)?
Gamma 1: gamma globulins
Gamma 2: CRP (acute phase reactant)
*CRP normally 2-3 mg/L but high level CRP elevation (>10 mg/L) usually indicates active inflammation such as collagen vascular disease, infection, etc and low level CRP elevation (3-10 mg/dL) is indicative of cellular stress and correlated with higher all cause mortality, poor outcome following cardiovascular events
What does it mean if you see a bimodal albumin peak on SPEP?
This is seen in heterozygotes for albumin alleles
No clinical significance
What pattern would you see on SPEP in a patient with nephrotic syndrome?
Loss of small serum proteins, particularly albumin, while large proteins are retained
Result is a decrease in all bands except alpha 2 (too big so it's retained)
What pattern would you see on SPEP in a patient with acute inflammation?
Increased alpha 1 and alpha 2
Normal-to-increased gamma globulins
What condition causes beta gamma briding on SPEP?
Indicative of cirrhosis cause by increased serum IgA
Additional features include hypoalbuminemia and blunted alpha 1 and alpha 2 bands
If the basal triglyceride level is elevated, what two clinical scenarios can cause this "pseudo-hypertriglyceridemia"?
Glycerol kinase def.
- (glycerol –GK-- > glycerol-3-phosphate)
These people aren't metabolizing TGs correctly
What is Friedewald equation (which you MUST KNOW for boards)?
LDL-C = TC – HDL-C – Tg/5* (*estimates VLDL-C)
If TG >400, it is invald!
What is Osler guy's helpful tip for remembering the different lipid disorders and what is elevated in them?
Increased TG: Types I (chylomicrons), IV (VLDL) and V (chylomicrons and VLDL)
Increased LDL: Types IIA (LDL)
Increased TG and LDL: Types IIB (LDL and VLDL) and III (IDL and remanant lipoproteins
What are the causes of type I hyperlipidemia (chyolmicronemia)?
Lipoprotein Lipase Deficiency: AR, rare, onset in infancy
Apo CII Deficiency: ligand for lipoprotein lipase, AR, rare, onset in childhood
SLE: acquired, autoantibody against lipoprotein lipase
What are the acquired and genetic causes of type IV hyperlipemia (increased VLDL)?
Insulin resistance: insulin receptor mutation, lipodystrophy
Familial combined hypertriglyceridemia
Inborn errors of metabolism:glycogen storage disease
Insulin resistance: increased VLDL production, decreased VLDL clearance, decreased activity of LPL (includes obesity, met syn, diabetes, Cushing, acromegaly, pheo, steroids)
What are the genetic causes of type IIA hyperlipemia and what particle is involved?
AR familial hypercholesteromia
Familial combined hyperlipidemia
What are the acquired causes of type IIA hyperlipidemia and what particle is involved?
Liver disease (e.g.,biliary tract disease*)
*in biliary tract disease, there is a lipoprotein called LpX which can falsely elevate both LDL and HDL
Renal disease (e.g., nephrosis)
Endocrine (hypothyroidism, diabetes mellitus)
Drugs - glucocorticoids, androgens
What are the causes of type IIB hyperlipidemia and what are the particles involved?
LDL & VLDL
Similar to HLP IIA
except: Familial hyperapo-Blipoproteinemia: HLP IIA only
(no incr. in VLDL)
What are the causes and particles involved in type III hyperlipidemia?
LDL and remnant lipoproteins
Happens if somone is homozygous for Apo E2/E2 AND has an environmental stressor that makes them hyperlipidemic such as:
- Renal disease
- Liver disease
What is the one genetic cause of low lipoproteins that you MUST KNOW for boards and how is it inherited, what particles are low and what are the clinical features?
Autosomal recessive failure of apo B production
- very low Tg & chol
- Very low: VLDL & LDL
HDL: preserved (lacks apo B)
developmental failure in infancy
What are the positive acute phase reactants?
What are the negative acute phase reactant?
Retinol binding protein (RBP)
What is the one body fluid where you can see a band in the pre-albumin peak on serum protein electrophoresis?
Also has a "2nd form" of beta which represents CSF transferrin
Hallmarks of CSF:
Double transferrin (B1) peak from asialated transferrin aka Tau protein
What proteins are represented in the prealbumin peak on SPEP?
Retinal Binding Protein (RBP) which transports VitA
Transthyretin which is a T4 transporter
*prealbumin is an indicator of nutritional status (half like 48 hours) and is a neg acute phase reactant
What are significant causes of decreases in RBP or
transthyretin (which are seen in the pre-albumin peak on SPEP)?
Transthyretin: malnutrition and nephrosis
*why would nephrosis be a prothrombotic state if you are losing proteins? You are also losing anti-coag like antithrombin 3
What are reasons for increased albumin on SPEP?
Prolonged tourniquet time
What proteins are represented by the beta region on SPEP?
Transferrin (beta 1)
LDL (beta 1-beta 2 interface)
IgA and C3 (beta 2)
What are the primary and secondary cause of hypogammaglobulinemia on SPEP?
XLA (Brutons agammaglobulinemia),
CVID (common variable immunodeficiency)
IgA (w/ IgG subclass: sinopulm/GI infections)
Hyper-IgM syndrome (CD40L--on T cells,CD40--on B cells, NEMO, AID)
Loss (nephrosis, burn, protein losing enteropathy)
Various forms of immunosuppresion
Chemotherapy for cancer
Irradiation for cancer
Immunosuppression for organ tx/AI Dz
Light chain disease