Pumping Iron and other proteins Flashcards
Iron study
test done secondary to a CBC, to investigate the cause of metabolic iron disorders, such as iron deficiency anemia or hemochromatosis
Serum iron normal
60-150 mcg/dL (varies w/age)
Serum iron
One single iron measurement is not always accurate as a solitary test to diagnose iron abnormalities and should be used in conjunction with other iron studies
Increased iron level
Beta- thalassemia Alcoholic cirrhosis-Damage to liver → increased ferritin levels (iron storage) High iron intake Hereditary hemochromatosis- HFE gene, impaired iron detection and regulation
Decreased Iron level
anemia chronic renal failure inadequate absorption increased loss increased demand
Serum ferritin test
Normal: 15-200 ng/ml Serum ferritin is the GOLD STANDARD in the diagnosis of iron deficiency anemia. Serum ferritin is helpful to distinguish between iron deficiency anemia (ferritin 10 ng/mL) Ferritin is an ACUTE PHASE REACTANT and can increase in liver disease, cancer, infection and inflammation
ferritin function
Ferritin molecules store thousands of iron atoms within their mineral core. When excess dietary iron is absorbed, the body responds by producing more ferritin to facilitate iron storage 15-20 % of body’s iron is stored within ferritin Ferritin is stored in liver , spleen , skeletal muscles, and bone marrow
increased ferritin
due to: hereditary hemachromatosis excess intake/poisoning chronic hepatitis
decreased ferritin
could be due to iron deficiency anemia
transferrin
are glycoproteins that are responsible for the transport of iron Transferrin can bind to two iron molecules. normally 33% of transferrin binding sites are filled. Transferrin transports iron from site of absorption to almost all tissues of the body.
Total Iron Binding Capacity
TIBC TIBC is the MAXIMUM amount of iron that serum proteins, mainly transferrin, can bind to. TIBC reflects the potential for iron binding if ALL of the binding sites on Transferrin were filled. is ordered in the evaluation of anemia and used with the serum iron and ferritin to evaluate and diagnose anemia. So a high TIBC indicates low iron presence
Increased TIBC
due to Iron deficiency anemia Pregnancy Oral Contraceptives Viral Hepatitis
decreased TIBC
Anemia of Chronic Disease Hemochromatosis Sideroblastic Anemia
Transferrin saturation test
Measurement of percentage of transferrin binding sites that are ACTUALLY bound by iron. An increase in the transferrin saturation represents an increase in iron absorption Normally, iron occupies 33% of the iron binding site on transferrin. % Transferrin Saturation = Serum iron/ TIBC
Increased transferrin saturation
Megaloblastic anemia Sideroblastic anemia Iron overload states Hemochromatosis
Decreased transferrin saturation
Iron deficiency anemia Chronic infection Malignancy Pregnancy Anemia of chronic disease
Iron deficiency anemia total phenotype
Decreased hemoglobin Decreased mean corpuscle volume MCV Increased RBC distribution width increased total iron binding capacity, TIBC decreased transferrin saturation decreased serum ferritin
Alpha/Beta Thalessemia total phenotype
decreased- normal hemoglobin decreased MCV normal RBC distribution width Normal TIBC normal transferrin saturation normal serum ferritin
Anemia of chronic disease total phenotype
decreased-normal hemoglobin decreased- normal MCV normal- increased RBC distribution width decreased TIBC decreased transferrin saturation decreased serum ferritin
Plasma components
is 55% of CBC of plasma, 91% of it is water 7% are blood proteins (albumin, globulin, fibrinogen) 2% are nutrients (AA’s, lipids, sugar) 1% is hormones and electrolytes
Albumin
Constitute approx. 50-60% of plasma proteins and are involved in maintenance of oncotic pressure, transportation of fatty acids, hormones, drugs and other substances.
Globulin
Constitute approx. 36% of plasma proteins Alpha and Beta globulins- produced by liver and involved in transportation or act as substrates Gamma globulins- produced by produced by lymphoid tissue- involved in immune system
Increased Serum albumin
in a CMP due to dehydration
Decreased serum albumin
in a CMP Due to: Liver disease Malabsorption/ Malnutrition Abnormal loss- renal disease, GI loss, Skin loss, severe burns
Prealbumin
Prealbumin is a much better assessment of patient’s nutritional status than albumin because it has a shorter half life and is more sensitive to rapid changes in nutrition. It is not included in a panel and must be ordered independently. The test may not be an accurate assessment of nutrition in patients with inflammation, infection or trauma.
Prealbumin increased
in pregnancy and non-hodgkins lymphoma
Prealbumin decreased
in Renal / Liver disease, Malabsorption or malnutrition, Crohn’s Disease, Low protein diet Severe illness, inflammation, or infection
Alpha-1-Antitrypsin
Plasma protein- is an Alpha-1 globulin. inhibits the action of many key enzymes that are released during inflammatory reactions in the lungs.
Decreased/defecient Alpha-1-antitrypsin manifestations
Chronic obstructive lung disease in adults- often before age 40 Prolonged Jaundice or hepatitis in Infants Liver dysfunction in children Portal hypertension Chronic Hepatitis Cirrhosis Hepatocellular Carcinoma
Ceruloplasmin
A plasma protein, is an alpha-2 globulin, is made in the liver, and is involved in copper transport in the body
Wilson’s disease
ceruloplasmin is low This results in impaired transport of copper and can lead to liver disease, which results from the excess of copper in the liver.
Increased ceruoplasmin
in infections also 1st trimester and some contraceptives
decreased ceruoplasmin
in wilson’s disease, liver failure, or hepatitis
Haptoglobin
a plasma protein–> hb scavenger Haptoglobin is produced in the liver and its function is to bind to free hemoglobin when RBCs are destroyed. Once haptoglobin is bound to hemoglobin, it transports hemoglobin back to the liver where the heme is converted to bilirubin. If there is increased RBC destruction, the haptoglobin becomes depleted and its levels decrease. Haptoglobin is very useful when looking for signs of hemolytic anemia.
decreased haptoglobin
in hemolytic anemia transfusion reaction artificial heart valve
complement protein
“complement” the action of antibodies to destroy and eliminate pathogens from the body. Generally, complement proteins are synthesized in the liver. C1-C9 Primary function of this cascade is to facilitate opsonization (Greek work for delicacy) of pathogens, making them “tastier” for neutrophils and allowing phagocytosis
Complement pathway
The ultimate goal and outcome of this pathway is to produce a membrane attack complex (MAC) through this pathway and insert itself into the membrane of the pathogen, causing its lysis and destruction. The most commonly measured complement proteins are C3 and C4
CH50
Total complement activity test This test is used to measure immune processes or to detect complement deficiency. The test quantitatively measure the ability of human serum to lyse sheep RBCs that have been coated with antibody (when 50% of cells lysed). The test results measure the amount of hemolysis that occurs. this is good as a screening test to look for an immune deficiency when a patient has had multiple unexplained viral or bacterial infections.
Decreased CH50
decreased complement activity
Ch50 = zero
one of pathway components absent
C3 and C4 compliment test, decreased
Systemic lupus erythematosus Bacterial infections
immunoglobins
are the most significant of the gamma globulins Also known as antibodies The variation in the heavy chains determines which of the five types of immunoglobulin
IgA
is the immunoglobulin usually found in secretions and along the mucosal epithelium. It is the defender of mucosal or epithelial surfaces against pathogens IgA present in saliva, tears, colostrum and mucus – think secretions which is why it is called secretory IgA
IgA defeciency
can be asymptomatic, or can cause freq URI, GI inflammation
IgE
Key factor involved in allergic reactions and parasitic infections IgE binds to mast cells and this initiates a chain of immune responses
Increased IgE
demonstrates that a person likely has allergies allergy test called Immunocap® which can determine specific IgE antibodies against allergens, such as dog, cat, mold, dust mites, foods etc.
IgG
Major antibody produced when an antigen is encountered IgG is most prevalent antibody in serum and has the longest half-life of all immunoglobulins = 23 days Can persist and be detected for life in some cases Mononucleosis Crosses the placenta so that the fetus can be protected from infection IgG comes in later = “I already Got Germs!” = I’m over it now. Or “IgG laGs behind.”
IgM
Initial antibody secreted after an immune challenge with half-life of 10 days IgM antibodies usually indicate recent infection IgM shows up first = “I get Meds!” = I’m sick now!
Protein electrophoresis
indicated in detecting some forms of cancer or pre-cancer, immune abnormalities, kidney or liver dysfunction
Multiple Myeloma
Neoplastic disorder which causes proliferation of a monoclonal immunoglobulin, usually IgG and IgA Clones of a single structurally-identical antibody multiplies rapidly and now becomes labelled the M protein (monoclonal protein)
Normal protein electrophoresis
Albumin is the biggest comonent, it is the pinky
then it is Alpha-1: alpha-1-antitrypsin
Alpha-2: haptoglobin
Beta 1/2: transferrin
Gamma (the thumb): Immunoglobulins are here.
IgM, migrates to beta region
IgA, b/w the beta and gamma peaks
IgG, appears throughout gamma
Protein electrophoreisis in multiple myeloma
Protein electrophoresis showing polyclonal gammopathy
Can indicate
•Liver disease- Cirrhosis or hepatitis
•Autoimmune disease- SLE (lupus), Rheumatoid arthritis
•Infection – HIV, hepatitis, osteomyelitis, endocarditis
•Hematologic disorders or malignancies- Non-Hodgkin’s Lymphoma, Sickle Cell Anemia, Thalassemia
•Non-hematologic malignancies
