CSP Exam Prep Course: Immunoglobulin Therapy

Comprehensive vocabulary flashcards covering immunology concepts, manufacturing processes, clinical indications, stabilizer safety profiles, dosing guidelines, and adverse event management for immunoglobulin (IVIg/SCIg) therapy.

Antigen

A foreign substance (such as chemicals, bacteria, viruses, or pollen) that induces an immune response.

Antibodies

Proteins produced by the immune system when harmful substances are detected; each type is unique to a specific antigen.

Autoantibodies

Antibodies produced by the immune system that mistakenly attack healthy cells.

Helper T-Cells

T-cells that stimulate B-cells to make antibodies and help killer cells develop.

Killer T-Cells

T-cells that directly kill host cells that have already been infected by a foreign invader.

B-Cells

White blood cells responsible for humoral immunity that produce antibodies to fight bacteria and viruses.

National Donor Deferral Registry (NDDR)

A centralized database where information is entered for blood and plasma donors who fail screenings and are permanently deferred.

Cohn Process

A plasma fractionation method using protein precipitation governed by ethanol concentration, ionic strength (addition of salts), temperature, and pH.

Kistler-Nitschmann Process

A cold-ethanol plasma fractionation technique developed as a customized adaptation of the Cohn process.

Primary Immunodeficiency (PI)

A group of over 550 rare, non-contagious, chronic disorders caused by hereditary or genetic immune system defects that lead to increased susceptibility to infections.

Immune Thrombocytopenic Purpura (ITP)

An autoimmune bleeding disorder characterized by low platelet levels; treated initially with corticosteroids or with IVIg dosed at $2\,\text{g/kg}$ divided over 2 days if platelet counts do not improve.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

A neurological autoimmune disorder causing symmetrical weakness and motor impairment through peripheral nerve inflammation and myelin sheath destruction; treated with an IVIg loading dose of $2\,\text{g/kg}$ over 2 to 5 days and maintenance of approximately $1\,\text{g/kg}$ every 3 weeks.

Dermatomyositis (DM)

An inflammatory myopathy causing muscle weakness/atrophy and skin abnormalities such as a reddish-purple butterfly rash on the face and scaling over knuckles, elbows, and knees; dosed at $2\,\text{g/kg}$ IV divided over 2 to 5 days every 4 weeks.

Multifocal Motor Neuropathy (MMN)

An immune-mediated motor nerve condition that causes slowly progressive muscle weakness, cramps, and atrophy primarily in the arms and legs; dosed at $0.5\text{--}2.4\,\text{g/kg}$ IV monthly based on response.

B-Cell Chronic Lymphocytic Leukemia (CLL)

A hematologic malignancy where the body loses infection-fighting capacity, often asymptomatic at diagnosis; treated with IVIg replacement at $400\,\text{mg/kg}$ IV every 3 to 4 weeks.

Kawasaki Syndrome

A pediatric condition featuring prolonged remittent fever, conjunctivitis, mucocutaneous inflammation, and coronary artery involvement; treated with IVIg $2\,\text{g/kg}$ as a single infusion within 10 days of fever onset alongside aspirin.

Maltose (as an Ig Stabilizer)

A sugar stabilizer in certain Ig formulations that can cause falsely elevated readings on blood glucose monitors using pyrroloquinoline quinone or glucose-dye-oxidoreductase.

D-sorbitol (as an Ig Stabilizer)

A product stabilizer that poses a severe toxicity risk in patients diagnosed with hereditary fructose intolerance.

Proline (as an Ig Stabilizer)

An amino acid stabilizer in certain immunoglobulin preparations that is contraindicated or poses a specific risk to patients with hyperprolinemia.

Sucrose (as an Ig Stabilizer)

A previously common stabilizer associated with a high incidence of renal complications, osmotic nephrosis, and acute tubular necrosis, leading to a Black Box Warning.

Normal Blood Osmolality

The physiological range of $275\text{--}295\,\text{mOsm/kg}$; deviation in hyperosmolar IVIg products increases risks of vein irritation, aseptic meningitis, and thromboembolic events.

Factor XIa

A clotting cascade impurity whose insufficient clearance during manufacturing leads to increased plasma viscosity, platelet aggregation, and thrombosis (warranting a Black Box Warning).

Anaphylactoid Reaction

A non-IgE mediated reaction causing gradual shortness of breath, chest tightness, and hypertension during the first half of an infusion, often triggered by complement-fixing IgG aggregates or prekallikrein activator (PKA).

Aseptic Meningitis

A severe post-infusion headache lasting hours to days secondary to meningeal inflammation, associated with high IVIg doses, rapid infusion rates, dehydration, or a history of migraines.

Hemolysis (in Ig Therapy)

The breakdown of red blood cells caused by anti-A or anti-B antibodies present in Ig products, presenting with dark urine, jaundice, and anemia within days to weeks; more common in non-O blood types.

Rigors

Sudden, uncontrollable shaking accompanied by fever and chills shortly after starting an infusion; managed by stopping the infusion and administering meperidine, supportive antipyretics, and antihistamines.

SCIg Dose Conversion Factor

The dose multiplier applied when switching from IVIg to subcutaneous Ig, requiring an adjustment factor of $1.37\text{--}1.53 \times \text{IV dose}$ depending on the brand and indication.

Recombinant Hyaluronidase

An enzyme utilized in facilitated SCIg (Hyqvia) to increase subcutaneous tissue permeability and allow high-volume infusion at a single site, commonly resulting in a transient, harmless 'pancake' swelling.