17 August 2011

Primary immune deficiency treatment

Immune deficiencies occur when an individual's ability to fight infectious disease is compromised or entirely absent. Patients who suffer from immune deficiencies experience recurrent infections, such as sinusitis and pneumonia.



Immune deficiencies occur when an individual's ability to fight infectious disease is compromised or entirely absent. Patients who suffer from immune deficiencies experience recurrent infections, such as sinusitis and pneumonia.

There are two main types of immunodeficiencies - primary immune deficiencies and secondary immune deficiencies.

Primary immune deficiencies are disorders that occur because part of the body's immune system does not function properly. These disorders are caused by intrinsic or genetic defects in the immune system. Therefore, individuals who have primary immune deficiencies are born with the disorder.

The World Health Organization (WHO) has identified nearly 100 primary immune deficiency diseases, including X-linked agammaglobulinemia (Bruton's Disease), common variable immune deficiency (CVID), and selective immunoglobulin A deficiency.

Many individuals affected by primary immune deficiency diseases require life-long therapies, such as intravenous immune globulin infusions, antibiotic therapies, or bone marrow transplantations.

Secondary immunodeficiencies are caused by factors outside of the body, such as chemotherapy treatment, radiation therapy, malnutrition, HIV infection, and diabetes. Diseases like leukemia and multiple myeloma (types of cancers) cause cancerous immune cells to infiltrate the bone marrow, which is responsible for producing immune system cells. Secondary immune deficiency also occurs among critically ill patients or the elderly.

Secondary immunodeficiencies usually resolve once the underlying illness is treated or the outside factor is eliminated. For instance, immune deficiencies caused by chemotherapy or radiation therapy generally resolve once the treatment is completed.


Primary immune deficiencies :

General: Currently, the only potential cure for primary immune deficiencies is a bone marrow transplant. The procedure has serious health risks, and it is only performed if the condition is life threatening. Other treatments, including intravenous immune globulin and interferon-gamma injections, may help boost the immune system and prevent recurrent infections.

Bone marrow transplant (BMT): A bone marrow transplant (BMT) can be performed in patients who have life-threatening immune deficiencies, such as severe combined immunodeficiency (SCID) and Wiskott-Aldrich syndrome. A successful BMT can permanently cure primary immune deficiencies.

However, not everyone is a candidate for a bone marrow transplant. The transplant must come from a donor whose body tissues are a close biological match to the recipient. Serious health risks are also associated with the procedure, as with any major surgery. Individuals who have weakened immune systems are at risk of developing graft-versus-host disease after surgery. This condition occurs when the transplanted bone marrow attacks the recipient's weakened immune system. Other recipients may experience transplant rejection, which occurs when the body's immune system attacks the donated organ.

In addition, patients who undergo organ transplants must take immunosuppressive medications for the rest of their lives in order to prevent their bodies from attacking the transplanted organs. The most common oral immunosuppressants prescribed in the United States include tacrolimus (Prograf©), mycophenolate mofetil (CellCept©), sirolimus (Rapamune©), prednisone (Prednisone Intensol©), cyclosoporine (Neoral©, Sandimmune© or Gengraf©) and azathioprine (Imuran©). Depending on the transplant, some patients may need to take different combinations and doses of these drugs. In general, patients are typically prescribed two to three medications for long-term immunosuppression.

Intravenous immune globulin (IVIG): Intravenous immune globulin (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. The beneficial effects of IVIG for the preventative treatment of patients with primary immunodeficiency syndromes are well established. IVIG has been shown to prevent severe lower respiratory tract infections, but not upper respiratory tract and non-respiratory infections in patients with common variable immune deficiency (CVID).

Immune globulin products contain sterile, purified immunoglobulin G (IgG). The products typically contain more than 95% unmodified IgG. The IgG antibodies are the smallest, but most abundant antibodies in the body, making up for 75-80% of all the antibodies in the body. Most IVIG preparations contain trace amounts of IgA, which can sensitize an IgA-deficient person 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 a severe allergic reaction called anaphylaxis after an IVIG infusion. These patients should receive the first infusion in the hospital under medical supervision.

The immune globulin is typically administered intravenously for about two to four hours a day for two to seven days. The patient usually receives another single dose every 10-21 days, or every three to four weeks depending on the condition. Patients typically start responding to treatment after eight days.

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©.

Interferon-gamma injections: Interferon-gamma injections have been used to treat certain immune deficiencies. Interferon-gamma solutions contain cytokines, which are natural chemicals produced by immune cells during an immune response. For instance, chronic granulomatous disease is caused by faulty phagocytes (white blood cells that ingest and destroy foreign substances that enter the body). These phagocytes can be activated with injections of a natural or synthetic product of immune cells called gamma interferon.

Secondary immune deficiencies :

General: Most secondary immune deficiencies resolve once the underlying illness is treated. The type of medication, dose and duration depend on the type and severity of the disease, as well as the patient's overall health. Immune deficiencies caused by chemotherapy or radiation therapy generally resolve once treatment is completed.

Infections: Infections may be treated with antibiotics (bacterial infection), antifungals (fungal infection) or antiviral (viral infection) medications.


Arginine (L-arginine) : Results of preliminary studies suggest that arginine supplementation may enhance the immune response elicited by the pneumococcal vaccine in older people. More studies are needed to confirm these results.

Astragalus : Astragalus has been suggested as an immune system stimulant in preliminary laboratory and animal research, and in traditional accounts. Reliable human studies are lacking, and high-quality human research is necessary before a firm conclusion can be drawn.

Beta-carotene : Preliminary research of beta-carotene for immune system maintenance or stimulation shows mixed results. Further research is needed before a conclusion can be drawn.

Cat's claw : A few early studies suggest that cat's claw may boost the immune system, including patients with HIV. Results from different studies have not been consistent; therefore, there is not enough information to recommend cat's claw for this use.

Copper : Copper is involved in the development of immune cells and immune function in the body. Severe copper deficiency appears to have adverse effects on immune function, although the exact mechanism is unclear.

Echinacea : Echinacea has been studied alone and in combination preparations for immune system stimulation (including in patients receiving cancer chemotherapy). It remains unclear if there are clinically significant benefits. Additional studies are needed in this area before conclusions can be drawn regarding its safety and effectiveness. Some studies have suggested that echinacea may increase white blood cell counts after X-ray treatment. However, studies have reported mixed results, and it remains unclear whether echinacea has benefits for this use.

Gamma linolenic acid (GLA) : Few clinical trials have investigated the effect of GLA on immune responses in healthy human subjects. Results from one randomized, clinical trial suggest that GLA, as blackcurrant seed oil, may offer some benefits. Further, well-designed clinical trials are required before definitive conclusions can be made.

Ginseng : Several studies suggest that ginseng can effectively enhance immune system function. Avoid ginseng if there is a known allergy to plants in the Araliaceae family. There has been a report of a serious life-threatening skin reaction, possibly caused by contaminants in the ginseng formulation.

There have been several studies investigating Cold-fX©, a patented American ginseng (Panax quinquefolius) extract, for potential immune system enhancement and increased resistance to colds and flu. Several studies suggest that Cold-fX© can effectively enhance the immune system. More studies are needed to determine whether Cold-fX© is a potentially effective preventative treatment for acute respiratory illness due to influenza and respiratory syncytial virus (an RNA-containing viruses that causes respiratory infections).

Avoid if allergic or hypersensitive to poly-furanosyl-pyranosyl-saccharides, or any ingredient found in Cold-fX©. Use cautiously with a known allergy hypersensitivity to Panax species, their constituents, poly-furanosyl-pyranosyl-saccharides specifically or to other members of the Araliaceae family. Anaphylaxis-like symptoms have occurred after ingestion of Panax ginseng syrup. Use cautiously with gastrointestinal disorders and with a history of diabetes. Avoid if pregnant or breastfeeding.

Goldenseal : Goldenseal has been suggested to be an immune system stimulant. However, there is little human or laboratory evidence in this area. More research is needed before a firm conclusion can be drawn.

Maitake mushroom : Animal and laboratory studies suggest that beta-glucan extracts from maitake may alter the immune system. However, no reliable studies in humans are available.

Massage : Preliminary evidence suggests that massage therapy may preserve immune function. Further research is needed before a firm conclusion can be made.

Meditation : Preliminary studies have reported increased antibody response after meditation. Further research is needed to confirm these findings.

Mistletoe : A few small trials found mistletoe to be promising as an immunostimulant in individuals with the common cold. Further research is needed to confirm these results.

Probiotics : Lactobacillus in fermented milk, low-fat milk or lactose-hydrolyzed low-fat milk may enhance immune function. Bifidobacterium may also be helpful in the elderly population. However, commercially produced yogurts may not yield similar benefits. There is some evidence that probiotics added during food preparation (e.g. waffles with Enterococcus faecium M-74 added) may enhance immune function. More studies are needed, particularly with yogurt, to provide concrete conclusions.

Vitamin A (retinol) : Vitamin A deficiency may compromise immunity, but there is no clear evidence that additional vitamin A supplementation is beneficial in patients who are not vitamin A deficient.

Vitamin B6 (pyridoxine) : Vitamin B6 is important for immune system function in older individuals. One study found that the amount of vitamin B6 required to reverse immune system impairments in elderly people was more than the current recommended dietary allowance (RDA). Well-designed clinical trials on vitamin B6 supplementation for this indication are needed before a firm conclusion can be made.

Vitamin E : Studies of the effects of vitamin E supplementation on immune system function have yielded mixed results. Further research is needed before a clear conclusion can be drawn.

Zinc : Zinc appears to be an essential trace element for the immune system, but research on the effect of zinc supplementation on immune function is scant and mostly focuses on patients with specific diseases. Zinc gluconate appears to exert beneficial effects on immune cells, improving CD3 and CD4 counts and increasing CD4/CD8 ratios in children. These cells are white blood cells that help fight off disease and infection. There are relatively few studies that examine zinc levels and the effects of zinc supplementation on the health of the elderly population. Further research is needed before a recommendation can be made.

Zinc is generally considered safe when taken at the recommended dosages. Avoid zinc chloride since studies have not been done on its safety or effectiveness. While zinc appears safe during pregnancy in amounts lower than the established upper intake level, caution should be used since studies cannot rule out the possibility of harm to the fetus.


Since primary immunodeficiencies are genetic, there is currently no known method of prevention. However, individuals can take steps to prevent or reduce the likelihood of developing infections, which are a result of their condition. Individuals should frequently wash their hands with soap and water, and avoid close contact with individuals who have contagious infections and diseases.

Intravenous immune globulin (IVIG) may help prevent infections in patients who have weakened immune systems.

Children who have primary immune deficiency diseases should not receive live virus vaccines, such as the oral polio, measles or chicken pox. This is because live viral vaccines can sometimes infect the recipient, especially if they are immunocompromised. In some cases, immunocompromised individuals can become infected after coming into close contact with a recently vaccinated individual.

Some secondary immune deficiencies can be prevented by avoiding or minimizing exposure to pathogens (disease-causing organisms). Individuals should regularly wash their hands with soap and water and avoid close contact with individuals who have contagious infections or diseases.


This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (

  • Immune Deficiency Foundation.
  • National Institute of Child Health & Human Development. Primary Immunodeficiency.
  • National Jewish Medical and Research Center.
  • Natural Standard: The Authority on Integrative Medicine.

Copyright © 2011 Natural Standard (

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Dr Morris is the Principal Allergist at the Cape Town and Johannesburg Allergy Clinics with postgraduate diplomas in Allergology, Dermatology, Paediatrics and Family Medicine dealing with both adult and childhood allergies. obesity and diabetes societies and runs a trial centre for new drugs.

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