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Intravenous immunoglobulin therapy

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Also listed as: WAS
Related terms
Background
Author information
Bibliography
Side effects
Preparations
Uses
Precautions
Monitoring ivig use

Related Terms
  • Antibodies, auto recessive, B-cells, bone marrow, bone marrow transplant, CBC, genetic disorder, immune system, immunodeficiency, inherited disorder, inherited immunodeficiency, leukocytes, leukemia, lymphoma, lymphocytes, malignancy, platelets, pneumonia, red blood cells, T-cells, thrombocytes, thrombocytopenia, tumor, WASP, white blood cells, Wiskott Aldrich syndrome, Wiskott-Aldrich syndrome protein, X-linked.

Background
  • Wiskott-Aldrich syndrome (WAS) is an inherited, immunodeficiency disorder that occurs almost exclusively in males. The recessive genetic disorder is caused by a mutation in the WAS (Wiskott-Aldrich syndrome) gene, which is an X-linked trait. The gene mutation leads to abnormalities in B- and T-lymphocytes (white blood cells), as well as blood platelet cells. In a healthy individual, the T-cells provide protection against viral and fungal infection, the B cells produce antibodies, and platelets are responsible for blood clotting to prevent blood loss after a blood vessel injury.
  • Individuals diagnosed with WAS suffer from recurrent infections, eczema and thrombocytopenia (low levels of platelets).
  • Before 1935, patients only lived an average of eight months. Today, patients usually live an average of eight years, according to a recent case study. The cause of death is usually attributed to extensive blood loss. However, cancer (especially leukemia) is common and often fatal among WAS patients.
  • The only possible cure for WAS is a bone marrow transplant. However, if a patient's family member is not a possible match for a bone marrow donation, patients may have to wait years for a potential donor. Other aggressive treatments may also increase a patient's life expectancy. For instance, one study found that patients who underwent splenectomy (removal of the spleen) lived to be more than 25 years old. The spleen may harbor too many platelets, and cause a decrease in the number of platelets in circulation. Antibiotics, antivirals, antifungals, chemotherapeutic agents, immunoglobulins and corticosteroids have also been used to relieve symptoms and treat infections and cancer associated with WAS.
  • Researchers estimate that about four people per one million live male births develop the disease in the United States.
  • The syndrome is named after Dr. Robert Anderson Aldrich, an American pediatrician who described the disease in a family of Dutch-Americans in 1954, and Dr Alfred Wiskott, a German pediatrician who discovered the syndrome in 1937. Wiskott described three brothers with a similar disease, whose sisters were unaffected.

Author information
  • This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography
  1. Binder V, Albert MH, Kabus M, et al. The genotype of the original Wiskott phenotype. N Engl J Med. 2006 Oct 26;355(17):1790-3.
  2. Jin Y, Mazza C, Christie JR, et al. Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hotspots, effect on transcription, and translation and phenotype/genotype correlation. Blood. 2004 Dec 15;104(13):4010-9. Epub 2004 Jul 29.
  3. Natural Standard: The Authority on Integrative Medicine. .
  4. St. Jude Children's Research Hospital. Inherited Immunodeficiencies: Wiskott-Aldrich Syndrome (WAS). .
  5. U.S. Immune Deficiency Foundation. The Wiskott Aldrich Syndrome. .

Side effects
  • The U.S. Food and Drug Administration (FDA) requires that all nonsteroidal anti-inflammatory drugs (NSAIDs) print serious warning labels that highlight the risk of potential heart-related side effects and stomach bleeding. The labels must also state that patients who have recently had heart surgery should not take NSAIDs.
  • The frequency and severity of side effects vary NSAIDs. The most common side effects include nausea, vomiting, diarrhea, constipation, decreased appetite, rash, dizziness, headache, and drowsiness. The most serious side effects include kidney failure, liver failure, ulcers, and prolonged bleeding after an injury or surgery. About 15% of patients on long-term NSAID treatment develop ulceration of the stomach and duodenum.
  • Phenylbutazone (Butazolidine®), a prescription NSAID (not available in the United States), is especially likely to cause serious side effects, including immunosuppression, which makes patients vulnerable to infection and disease.
  • NSAIDs may also cause fluid retention, which may lead to edema (fluid in the body tissues). Edema often causes swelling in the arms and legs.
  • Patients who have asthma may develop shortness of breath and are at an increased risk of experiencing a non-allergic drug reaction to NSAIDs.
  • Patients who are allergic to one type of NSAID are likely to experience a similar reaction to other types of NSAIDs.

Preparations
  • Intravenous immunoglobulin (IVIG) consists of the antibodies extracted from pooled blood donations from 3,000-10,000 healthy donors. In some instances, blood from as many as 100,000 donors is used.
  • Immune globulin products contain sterile, purified immunoglobulin G (IgG). The products typically contain more than 95% IgG. Most preparations contain trace amounts of IgA, which may sensitize an IgA-deficient patient during long-term treatment. Patients who suffer from severe, recurrent viral or bacterial respiratory tract infections or have isolated IgA deficiency (and additional IgG2 and IgG4 deficiency) may develop severe anaphylactic reactions after IVIG treatment, which may be life threatening. Therefore, these patients should receive the first infusion in the hospital under medical supervision.
  • Immune globulin products also contain small amounts of cytokines, CD4 cells, CD8 cells, and human leukocyte antigens (HLA). Cytokines are produced by immune cells to stimulate the immune response. CD4 cells detect antigens in the body. CD8 cells detect and destroy body cells that are infected with a bacteria, virus or fungi. HLA molecules help the body's immune system distinguish between self and non-self (foreign or invading) substances.
  • IVIG is available in different concentration (strengths). The U.S. Food and Drug Administration (FDA) has approved Gammagard S/D®, Gammar-P IV®, Gamimune-N®, Iveegam®, Polygam® S/D, Sandoglobulin® Venoglobulin-I®, Venoglobulin-S®, Carimune/Panglobulin®, Gamunex®, and Baxter AG®.

Uses
  • NSAIDs are primarily used to reduce inflammation, pain (mild to moderate), and low-grade fever. The anti-inflammatory effects may take anywhere from a few days to three weeks to initiate.
  • Specific uses include the treatment of headaches, arthritis (including osteoarthritis and rheumatoid arthritis), back pain, sciatica (pain extending from lower back down the leg), pain after surgery, fever, dental pain, kidney stone pain, sports injuries, menstrual cramps, tendonitis (inflamed tendons), and other painful conditions (especially where there is inflammation).
  • NSAIDs are also added to many cold and allergy drugs to relieve symptoms such as minor aches and pains, headaches, and fevers.

Precautions
  • Avoid with a history of peptic ulcers because nonsteroidal anti-inflammatory drugs (NSAIDs) increase the risk of developing duodenal or stomach ulcers.
  • Avoid with history of kidney or liver damage because NSAIDs may increase the risk of developing kidney or liver failure.
  • Avoid with indigestion or heartburn because NSAIDs may cause upset stomach or indigestion.
  • Avoid in patients who consume three or more alcoholic drinks a day because heavy drinkers may have an increased risk of developing liver damage and stomach bleeding from NSAIDs.
  • Do not take antacids or eat fatty foods with COX-2 inhibitors because they limit the body's ability to absorb the medication.
  • Do not take traditional NSAIDs (prescription or over-the-counter) with celecoxib (Celebrex®), the only selective COX-2 inhibitor approved by the U.S. Food and Drug Administration (FDA).
  • Use cautiously with asthma because NSAIDs may cause shortness of breath. Also, asthma patients are at an increased risk of experiencing a non-allergic drug reaction to NSAIDs.
  • Use cautiously with bleeding disorders because NSAIDs may cause prolonged bleeding after an injury or surgery.
  • Use cautiously if pregnant or breastfeeding. NSAIDs may damage the heart or blood flow of the fetus or newborn baby if they are taken regularly during the last few months of pregnancy. Some animal studies have shown that if NSAIDs are taken late in pregnancy, they may increase the length of pregnancy, prolong labor, or cause complications during delivery. Pregnant mothers should consult a qualified healthcare provider before taking NSAIDs. Although NSAIDs have not been shown to cause medical problems in breastfeeding babies, diclofenac (Cataflam® Voltaren®-XR), diflunisal (Dolobid®), fenoprofen (Nalfon®), flurbiprofen (Ansaid®), meclofenamate (Meclofenamate sodium®), mefenamic acid (Ponstel®), naproxen (Aleve®), piroxicam (Feldene®), and tolmetin (Tolectin® or Tolectin® DS) can pass into breast milk. Caution is warranted.

Monitoring ivig use
  • Patients receiving intravenous immune globulin (IVIG) therapy should be closely monitored. During a physical examination, healthcare providers should obtain a complete medical history, including whether a patient has ever had liver or kidney disease or reactions to blood products or transfusions.
  • Kidney function tests should be performed because there have been reports of kidney failure associated with IVIG.
  • A complete blood count (CBC) should be conducted because patients who receive IVIG have a risk of developing hyperproteinemia (high levels of protein in the blood), increased serum viscosity and pseudohyponatremia (low levels of sodium in the blood), thrombosis (blood clots deep in the legs), transient serum sickness, transient neutropenia, or severe cutaneous vasculitis.
  • Patients should be tested for hepatitis because there have been reports of hepatitis transmission through IVIG.
  • Immunoglobulin levels should be tested to make sure the patient does not have IgA deficiency. Individuals who are IgA deficient should not receive IVIG because they may experience a severe allergic reaction called anaphylaxis.
  • Rheumatoid and cryoglobulin levels should be tested because IVIG may cause blood disorders.

Copyright © 2011 Natural Standard (www.naturalstandard.com)


The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.

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