Global

Immunodeficiencies

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CSL Behring therapies are effective in treating a number of serious conditions including:

  • Primary immunodeficiencies (PIDs) constitute a group of disorders, usually genetic, that cause a malfunction in all or part of the immune system, thereby rendering the patient unable to fight off infections caused by everyday germs.i Collectively, PIDs affect an estimated 10 million people worldwide.ii The incidence is estimated to be 1 in 10,000.iii
  • Secondary immunodeficiencies can be caused by several conditions, including: multiple myeloma, chronic lymphocytic leukemia, children with congenital acquired immunodeficiency syndrome (AIDS), and recurrent infections.
  • Idiopathic thrombocytopenic purpura (ITP) is the condition of having a low platelet count. Most cases are related to the presence of antibodies against blood platelets. Very low platelet counts can lead to unusual susceptibility to bleeding as well as to red or purple skin discolorations.
  • Guillain-Barré (ghee-yan bah-ray) Syndrome (GBS), also called acute inflammatory demyelinating polyneuropathy and Landry's ascending paralysis, "is an inflammatory disorder of the peripheral nerves those outside the brain and spinal cord. It is characterized by the rapid onset of weakness and, often, paralysis of the legs, arms, breathing muscles and face. GBS is the most common cause of rapidly acquired paralysis in the United States today, affecting one to two people in every 100,000." GBS/CIDP Foundation website
  • Kawasaki disease (KD), also known as Kawasaki syndrome, is a serious illness characterized by inflammation of blood vessels throughout the body that primarily affects young children and infants. Kawasaki disease is the leading cause of acquired heart disease in children. Although about 80 percent of patients are under five years of age, older children and teenagers can also get KD, but this is uncommon. KD is more common in boys than girls, and the majority of cases are diagnosed in the winter and early spring. It is not contagious.
  • Chronic inflammatory demyelinating polyneuropathy (CIDP) "is a rare disorder of the peripheral nerves characterized by gradually increasing weakness of the legs and, to a lesser extent, the arms. It is caused by damage to the covering of the nerves, called myelin. It can start at any age and in both genders. Weakness occurs over two or more months." GBS/CIDP Foundation website
  • Multifocal Motor Neuropathy (MMN) "is an acquired, chronic but treatable condition affecting multiple motor peripheral nerves that connect the spinal cord with the muscles. Damage to these nerves by the patient's immune system results in muscle weakness most often in the arms with minimal or no sensory changes. It develops in a chronically progressive or step-wise manner and over time leads to wasting or atrophy of the muscles controlled by the involved nerve. Males are affected with MMN almost three times as frequently as females and experience an earlier onset with a peak incidence between 50 and 60 years of age." GBS/CIDP Foundation website
  • Myasthenia Gravis (MG) "The term 'Myasthenia Gravis' comes from the Greek and Latin words meaning 'grave muscular weakness.' The most common form of MG is a chronic autoimmune neuromuscular disorder characterized by fluctuating weakness of the voluntary muscle groups. The prevalence of MG in the United States is estimated to be about 20/100,000 population. However, MG is probably under-diagnosed and the prevalence may be higher. Myasthenia Gravis occurs in all races, both genders, and at any age. MG is not thought to be directly inherited nor is it contagious. It does occasionally occur in more than one member of the same family." Myasthenia Gravis Foundation of American website
  • i Lindegren ML, Kobrynski L, Rasmussen SA, et al. Applying Public Health Strategies to Primary Immunodeficiency Diseases: A Potential Approach to Genetic Disorders. MMWR. 2004; 53(RR-1):1-29.
  • ii Jeffrey Modell Foundation. Primary Immunodeficiency Resource Center. Available on http://www.info4pi.org/aboutPI/, last accessed September 2009.
  • iii Dube D. The challenge of immunodeficiency disorders. Postgraduate Medicine 2002.

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