An essential protein found in human plasma that accounts for about 50 to 60 percent of plasma proteins.2 Each molecule is composed of three units, or domains, which function together to give albumin its unique binding properties.3 It is the primary agent responsible for maintaining osmotic pressure of the blood, and for transport of fatty acids, hormones, enzymes, and therapeutic drugs.4 Albumin is primarily responsible for 75 to 80 percent of plasma's normal colloid oncotic pressure.2 Albumin supplementation restores intravascular volume in an attempt to maintain cardiac output and colloid osmotic pressure. When plasma volume is drastically reduced, albumin may help restore the losses.2
Allogeneic bone marrow transplantation
Donor marrow received that is antigenically distinct.5
Alpha-1 antitrypsin deficiency (AATD)
A genetic condition in which AAT is not released from the liver into the blood. As a result, the affected person does not have enough AAT throughout the body. Some individuals with AAT deficiency are not affected, but other individuals develop liver problems like cirrhosis and/or lung problems like emphysema.6
Alpha-1 antitrypsin (AAT)
A protein made in the liver that is normally released into the blood. AAT functions to protect delicate body tissues from being destroyed by tissue-digesting enzymes that can damage cells.6
Alzheimer’s disease
Characterized by multiple symptoms, spanning from some memory loss to impairment of daily living. 5
Antibody
A protein produced by blood plasma cells that binds specifically to substances foreign to the body. Once bound, the antibody can lead to the destruction or inactivation of the foreign substance.5
Antithrombin III (AT III)
A protein of normal plasma and extravascular sites. It inactivates thrombin and inhibits certain coagulation factors.5
B-cell chronic lymphocytic leukemia (B-CLL)
Cancer of the white blood cells (WBC, lymphocytes); it causes a slow increase in the number of WBCs called B cells in the bone marrow.5 Symptoms can include hypogammaglobulinemia and/or recurrent bacterial infections.7
Coagulation
Another word for clotting of the blood. It is the process in which liquid blood is changed into a jelly-like substance to seal an injured blood vessel.1
Common variable immunodeficiency (CVID)
A disorder characterized by low levels of serum immunoglobulins (antibodies) and an increased susceptibility to infections. The genetic causes of the low levels of serum immunoglobulins are not known in most cases. It is a relatively "common" form of immunodeficiency, but the degree and type serum-immunoglobulin deficiency as well as the clinical course is "variable" from patient to patient.8
Coumarin-induced skin necrosis
A microthrombotic lesion found in central fatty areas of the body. Patients with congenital protein C deficiency are particularly susceptible. 9-11
Guillain-Barré Syndrome
A rare autoimmune disorder in which the peripheral nervous system is attacked by the body's immune system. Initial symptoms include degrees of weakness or tingling sensations in the legs, and can spread to the arms and upper body. In life-threatening cases these symptoms can intensify to near-total paralysis and may require a respirator to assist with breathing. However, most patients recover from even severe cases of Guillain-Barré syndrome, but may continue to have some degree of weakness.12
Human immunodeficiency virus (HIV)
A virus that kills or damages cells of the body's immune system.
Humoral immunity
Refers to antibody production and multiple related processes as well as antibody effector functions.13
Hypogammaglobulinemia
Lower than normal levels of gamma globulins or immunoglobulins (or antibodies) in the blood.8
Hypovolemia
An abnormal decrease in blood volume or, strictly speaking, an abnormal decrease in the volume of blood plasma. From hypo- + volume + emia (blood).14
Immune thrombocytopenic purpura (ITP)
An autoimmune disease characterized by a low blood platelet count.15 Patients may experience bruising, nose bleeds, or bleeding gums when platelet counts are low.
Immunoglobulins (Ig)
Any of the structurally related glycoproteins that function as antibodies. They are divided into 5 classes on the basis of structure and biological activity: IgA, IgD, IgE, IgG, and IgM. IVIg consists mainly of IgG.
Intravenous
A route for administration of medications and fluids via the bloodstream through a vein.1
Intravenous immunoglobulin (IVIg)
Glycoproteins that function as antibodies administered via the bloodstream.1
Kawasaki disease
A disease whose symptoms include coronary artery aneurysms and other coronary artery abnormalities.16
Kawasaki syndrome
A disease whose symptoms include coronary artery aneurysms and other coronary artery abnormalities.17
Multifocal motor neuropathy (MMN)
A rare slow-progressing disease characterized by asymmetrical muscle weakness and muscle atrophy with autoimmune-mediated pathogenesis.1
Post-exposure prophylaxis
A medical intervention to prevent tetanus infection in individuals who are at risk, usually through wound contamination.18,19
Primary immunodeficiencies (PID)
Immunodeficiencies intrinsic to cells and tissues of the immune system, and not due to another illness, medication or outside agent damaging the immune system. IVIg therapy is used to treat PIDs associated with defects in humorol immunity (ie, a lack of antibody production). These include, but are not limited to, common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiency. IVIg therapy is not effective for some types of PID, such as selective IgA deficiency or complement deficiencies.20 To date there are over 150 PIDs classified by the World Health Organization (WHO).21,29
Purpura fulminans
Bruising, necrosis and discoloring of the skin as a result of severe clotting of small blood vessels in the skin. 22,23
Secondary immunodeficiencies (SID)
Patients with SID's can be susceptible to a weakening of their immune systems and as a result recurrent infections due to low antibody levels. A number of conditions such as malignancies, particularly those of the haematopoietic and lymphoreticular systems, metabolic disease, malnutrition, burns or severe infection can also cause defective immune function and poor antibody response. 1,2 These conditions include but are not limited to chronic lymphatic leukemia (CLL) and myeloma (MM).
Severe combined immunodeficiency (SCID)
a genetic disorder in which both B cells and T cells of the adaptive immune system are crippled, due to a defect in one of several possible genes. SCID is a severe form of heritable immunodeficiency.25
Severe congenital Protein C deficiency
A rare genetic disorder that interferes with Protein C production, resulting in either Protein C deficit or malfunction.
Subcutaneous
Administration of a product under the skin.1
Tetanus, clinically manifested tetanus:
An acute and often fatal infectious disease caused by an anaerobic spore-forming bacillus which often enters the body through contaminated puncture wounds. Other portals of entry can include burns, surgical wounds, and cutaneous ulcers. Clinically manifested tetanus presents as local muscle spasms in areas contiguous to an infected wound.27
Venous thrombosis
Blood clot in the vein.22,23
Wiskott-Aldrich syndrome
An inherited defect of the immune system, caused by a mutation in the WAS gene located on the X chromosome.28
X-linked agammaglobulinemia
A PID disorder in which males are susceptible to bacterial infection, due to a mutation in the Btk gene which leads to a lack of antibody production.5 The disorder is X-linked as it is on the X chromosome, and is almost entirely limited to the sons of asymptomatic female carriers.8
Srinivasan AF, Rice L, Bartholomew JR. Warfarin-induced skin necrosis and venous limb gangrene in the setting of heparin-induced thrombocytopenia. Arch Intern Med. 2004;164:66-70
Broekmans AW, Bertina RM, Loeliger EA, Hofmann V, Klingemann HG. Protein C and the development of skin necrosis during anticoagulant therapy [letter]. Thromb Haemost. 1983;49:251
Moritz B, Rogy S, Tonetta S, Schwarz HP, Ehrlich H and the CEPROTIN Study Group. Efficacy and safety of a high purity protein C concentrate in the management of patients with severe congenital protein c deficiency. In: Scharrer I, Schramm W, ed. 31st Hemophilia Symposium Hamburg 2000. Berlin Heidelburg: Springer-Verlag; 2002:101-109
KIOVIG Human Normal Immunoglobulin (IVIg) [package insert]. Wallisellen, Switzerland. Baxter Healthcare SA; March 2006
National Human Genome Research Institute (NHGRI) Web site. Learning about severe combined immunodeficiency (SCID). Available at: http://www.genome.gov/13014325
Bongard FS, Sue DY. Current Critical Care Diagnosis & Treatment. Edition: 2. New York: Lange Medical Books/McGraw-Hill, 2002. ISBN 0838514545, 9780838514542
Aldrich RA, Steinberg AG, Campbell DC. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea. Pediatrics. 1954;13(2):133–139
Geha RS MD, Notarangelo LD MD, Casanova JL MD, et al. Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol; 2007; 120(4): 776-794.
