protein disorders

Monoclonal gammopathy

Polyclonal gammopathy- infection

Beta-Gamma Bridging- cirrhosis of the liver

Hypogammaglobulinemia- immunocompromised

A1AT/AAT deficiency

Nephrotic Syndrome

Acute Inflammation/Acute Phase proteins

Chronic Inflammation- chronic infection, autoimmune, and malignancy

Severe Liver Disease- hepatic disease/ liver failure

Bisalbuminemia (rare)

CSF electrophoresis

Presence of oligoclonal banding, aids to diagnose multiple sclerosis

Immunofixation

Used to characterize monoclonal gammopathy

Identifies light and heavy chains causing the M- spike

Hypoproteinemia

Caused by renal or hepatic disease, leakage from tissues or GI circulation, bleeding, malnutrition/malabsorption

Hyperproteinemia

Caused by dehydration and excessive protein production

Hemoglobin

Molecule in RBCs that binds to O2 to deliver to tissues

Albumin

Negative APR, most abundant transport protein in serum

Microalbumin/albuminemia

Small amounts of albumin in the urine, have a specific dipstick to test albumin/creatinine ratio

Prealbumin

Negative APR, thyroxine binding protein transporting T3 and T4

Hallmark of CSF electrophoresis

α-1 antitrypsin (AAT/A1AT)

Positive APR that inactivates trypsin like enzymes

Inherited and linked to pulmonary emphysema and cirrhosis

α-1 fetoprotein (AFP)

Positive APR used in prenatal testing

Increase indicates twins or neural tube defects

Decrease indicates risk of Down syndrome

α-1 acid glycoprotein (AAG)

Positive APR that inactivates progesterone and mediates spingomyelin metabolism

α-2 macroglobin

Positive APR that binds to inhibit proteases and gets rid of blood trash in the spleen

Increased in renal disease

α-2 Haptoglobin

Positive APR that binds to hemoglobin to prevent loss into the urine during a hemolytic episode

α-2 ceruloplasmin

Positive APR aiding in copper storage and transport

Decreased levels found in Menkes and Wilsons

Transferrin

Negative APR that binds and transports ferric iron

Tau protein in CSF is a hallmark of double beta peak

Fibrinogen

Positive APR that should only be seen in plasma and decreases coagulation activation

β2 microglobulin (B2M/BMG)

Urinary used to measure renal tubular function and prognostic factor in multiple myeloma

Complement

Group of proteins that increase inflammation in immune response

Decreased in lupus and malnutrition

C- reactive protein (CRP)

Non-specific APR and an independent risk factor in cardiovascular disease

Troponin (Tn)

Cardio specific protein elevated in myocardial infarction

Interference include fibrin and heterophile antibodies

β-type Natriuretic Peptide (BNP)

Synthesis correlates directly with ventricular wall tension and is elevated in patients with congestive heart failure

Gamma globulins

Synthesized by plasma cells from B-lymphocytic lineage

Familial Lipoprotein Lipase Deficiency

Decreased LPL enzyme leading to decreased metabolism of chylomicrons to VLDL

Presents as pancreatitis

Familial apo C-II deficiency

Decreased LPL enzyme activation leading to decreased metabolism of chylomicrons to VLDL

Familial Hypertriglyceridemia

Inhibited LPL enzyme leading to decreased metabolism of chylomicrons to VLDL

Familial Dysbetalipoproteinemia

Alteration of apo E structure leads to impaired binding to LDLR leading to decreased metabolism of chylomicron/ VLDL remnants

Familial Hypercholesteremia

Absent or reduced LDLR on cells so LDL is not removed from circulation

Myocardial infarction early in life

Familial Combined Hyperlipidemia

Increased apo B-100 production leading to increased production of VLDL and LDL

Tangiers Disease

Defect in HDL pathway leading to decreased HDL production (increases foam cells)

Hyperplasticity orange tonsils

Arteriosclerosis

Thickening of blood vessels due to buildup of cholesterol plaques

Atherosclerosis

Large arteries are hardened and narrowed often from hyperlipidemia