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Immune Disorders

 

The immune system plays a key role in numerous conditions. In so-called immune deficiencies, patients are prone to severe infections due to a lack of naturally occurring protective antibodies (immunoglobulins). These people need to have their missing immunoglobulins replaced in order to protect themselves against infections so they can lead a normal life.

 

Conditions where the immune system is out of balance are generally referred to as immune-mediated diseases. Of these, auto-immune diseases are particularly well known. Such conditions may lead to various other diseases, including bleeding disorders with a reduced number of blood platelets known as immune thombocytopenic purpura (ITP), inflammatory neurologic conditions such as Guillain-Barré Syndrome (GBS), chronic demyelinating polyradiculoneuropathy (CIDP). Many other organ systems can also be a target of an immune-mediated disease.

 

Immunotherapy treats deficiencies and immune mediated diseases by inducing, enhancing or suppressing an immune response through immunomodulation or immunoglobulin replacement therapy.

 

Conditions commonly treated with immunotheraputics and other products

Primary Immunodeficiency (PID)

 

The World Health Organization recognizes more than 150 primary immunodeficiency diseases, defined as a group of diverse disorders caused by abnormalities of the immune system. Some are relatively common, others are quite rare; some affect a single cell within the immune system, while others may affect one or more components of the system.1

 

Primary Immunodeficiencies (PIDs) result from defects inT-lymphocytes, B lymphocytes, NK cells, phagocytic cells or the complement system. Most are inherited diseases and may run in families, such as X-linked agammaglobulinemia (XLA) or Severe Combined Immunodeficiency (SCID). Other primary immunodeficiency, such as Common Variable Immunodeficiency (CVID) and Selective IgA Deficiency are not always inherited in a clear-cut or predictable fashion. In these disorders the cause is unknown but may involve an interaction of genetic and environmental factors. 2

 

PID symptoms are normally characterized by recurrent, persistent and severe infections, as shown in examples in the 10 warning signs below, created by the Jeffrey Modell Foundation and the American Red Cross:

  1. Four or more new ear infections within a year
  2. Two or more serious sinus infections within a year
  3. Two or more months on antibiotics with little effect
  4. Two or more pneumonias within a year
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent, deep skin or organ swelling
  7. Persistent thrush in mouth or fungal infection on skin
  8. Need for intravenous antibiotics to clear infections
  9. Two or more deep-seated infections including septicemia
  10. A family history of primary immunodeficiency

1. Immune Deficiency foundation (IDF) website

2. Immune Deficiency Foundation patient and family handbook

 

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

 

Myeloma and Chronic Lymphatic Leukaemia (CLL) with Severe Secondary Hypogammaglobulinaemia

 

Myeloma

 

Myeloma is a cancer that begins in plasma cells, a type of white blood cell 3. Early symptoms may vary and therefore should be diagnosed by health specialists only.

 

Chronic lymphatic leukemia (CLL) with severe secondary hypogammaglobulinaemia. CLL is a type of cancer of the blood and bone marrow caused by a slow increase in the number of white blood cells (B lymphocytes or B cells) in the bone marrow 4. Symptoms may vary and therefore should be diagnosed by health specialists only.


3. Website of the national institute of health USA - Myeloma

4. Website of the national institute of health USA - CLL

 

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

Immune Thrombocytopenic Purpura (ITP)

 

Immune Thrombocytopenic Purpura (ITP) is an autoimmune disease characterized by brief or persistent decreases in platelet count and increased risk of bleeding 5. Such bleeding can be life-threatening, for example, in the case of cerebral bleeds.

 

Symptoms can include 6:

  1. Abnormally heavy menstruation
  2. Bleeding into the skin, causing a skin rash that looks like pinpoint red spots (petechial rash)
  3. Easy bruising
  4. Nose bleeding or bleeding in the mouth

5. Robak, T et al.: Efficacy and safety of a new intravenous immunoglobulin 10% formulation . Hematology, October 2010

6. Website of the national institute of health USA - ITP

 

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

Kawasaki Disease

 

Kawasaki Disease is an autoimmune disease that usually occurs in young children and results in several immunological abnormalities7, which may damage the heart.

 

Symptoms can include8:

  1. High fever
  2. Swollen lymph nodes in the neck
  3. A rash on the mid-section and genital area
  4. Red, dry, cracked lips and a red, swollen tongue
  5. Red, swollen palms of the hands and soles of the feet
  6. Redness of the eyes

 


7. Ferencik, M et al.: Dictionary of Immunology (2000). Bratislava: SAP 163

8. Website of national institute of health US - Kawasaki

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

Guillain-Barré Syndrome (GBS)

 

Guillain-Barré Syndrome (GBS) is an autoimmune disease of the peripheral nerve system (autoimmune neuropathy) 9.

 

The first symptom is usually weakness or a tingling feeling in the legs. The feeling can spread to the upper body. In severe cases, patients become almost paralyzed. This is life-threatening, and patients may need a respirator to breathe. Symptoms usually worsen over a period of weeks, then stabilize. Most people do recover, with recovery taking a few weeks to a few years 10.


9. Hughes, R.A.c. et al.: Clinical applications of intravenous immunoglobulins in neurology. Clinical and Experimental Immunology, 158 (1):34-42

10. Website of the national institute of health USA. - GBS

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

 

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an autoimmune disease that targets and breaks down the protective coating (called myelin sheaths)  of the peripheral nerves (chronic inflammatory neuropathy). 11

 

Initial symptoms include weakness of the limbs. Sensory symptoms are common, such as tingling and numbness of hands and feet, but usually the loss of motor symptoms is more common.12 


11. Hughes, R.A.C. et all.: Clinical applications of intravenous immunoglobulins in neurology. Clinical and Experimental Immunology, 158 (1):34-42

12. Emedicine website

 

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

Bone Marrow Transplantation

 

Bone marrow transplantation using donor stem cells from the donor’s healthy bone marrow or blood.

Note: this content is for information only. For diagnosis and treatment, please consult your physician.

 

“...high-purity products for treating immune-mediated diseases and deficiencies”