Case Study
Lymphedema, also known as lymphatic obstruction, is a condition of localized fluid retention caused by a compromised lymphatic system. The lymphatic system (often referred to as the body's "second" circulatory system) collects and filters the interstitial fluid of the body. Lymphedema has been barely recognized as being a serious health problem; however, this is slowly changing due to education and awareness. The danger with lymphedema comes from the constant risk of developing an uncontrolled infection in the affected limb. Still, physicians and medical staff who practice in fields where this disease is uncommon may fail to correctly diagnose the condition due to the apparent lack of information regarding this disease.

In extreme cases, the condition is also know as elephantiasis.

ALPS – Autoimmune Lymphoproliferative Syndrome ALPS is a rare disease that can isn’t age specific. Problems from this disorder were not studied until 1990 when new symptoms began to arise that didn’t fit with any previous known disorders. It was named in 1995.

What is ALPS?

• Autoimmune – disease of the immune system
• Lymphoproliferative – describes the unusually high numbers of lymphocytes (white blood cells) stored in the lymph nodes and spleen of people with ALPS

• ALPS is not contagious.
• ALPS is a disorder developed in childhood. Problems appear to improve with age.

Common autoimmune problems in ALPS patients:

• Very low erythrocyte (red blood cell) counts, causing fatigue

• Very low platelet counts (immune-mediated thrombocytopenia), causing bruising, nosebleeds, and possibly petechiae. It also puts you at
• Risk for hemorrhageVery low white blood cell counts (autoimmune neutropenia), creating risk for infection

Understanding ALPS and Management of ALPS: In ALPS, apoptosis doesn’t work as it should. After an infection is gone, lymphocytes continue working. As a result, B and T cells accumulate in the lymph nodes, spleen, and liver. In general the extra T cells are fine, but the B cells are the ones that sometimes make a mistake. The B cells may make antibodies for platelets, erythrocytes, or other cells, instead of antibodies against germs. Since these antibodies stick to the platelets, erythrocytes, or other cells, and get stuck in the spleen, it has to work twice as hard to filter them out. This is why a symptom of ALPS can be an enlarged spleen. Though there is no cure for ALPS, however there are effective ways to manage:

• The first step is diagnosis which usually takes a while since most doctors don’t recognize the symptoms.
• The more you educate yourself about ALPS and its symptoms, the better you will be at managing it.
• Swelling of the lymph nodes and the problems with apoptosis are currently the untreatable aspects
• As symptoms and signs arise, inform your doctor in order to best manage ALPS.
• Virtually everyone with ALPS has an enlarged spleen. A splenectomy is a treatment option for this, though it poses both risks and benefits and must be carefully considered with your physician. A splenectomy is usually only considered if the patient is severely anemic.
• The first line of treatment for autoimmune episodes are steroids (the most common being prednisone).

Genetics:
Children can inherit ALPS from one parent. In more than 83 percent of the cases, an alteration in a gene that encodes for Fas has been found, which causes the gene to produce abnormal Fas protein. Though this mutation clearly leads to ALPS, it is not the only explanations. Further research is needed to identify other factors, gentic and non-genetic, that may contribute to developing ALPS.