Autoimmune lymphoproliferative syndrome (ALPS) is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis.It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T lymphocytes and Fas-ligand on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell apoptosis. Patients with ALPS have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation, autoimmune disease, and secondary cancers. Signs and symptoms
All people with ALPS have signs of lymphoproliferation, which makes it the most common clinical manifestation of the disease. The increased proliferation of lymphoid cells can cause the size of lymphoid organs such as the lymph nodes and spleen to increase (lymphadenopathy and splenomegaly, present in respectively over 90% and over 80% of patients). The liver is enlarged (hepatomegaly) in 30–40% of patients.Autoimmune disease is the second most common clinical manifestation and one that most often requires treatment. The most common autoimmune presentations include autoimmune cytopenias, which can be mild to very severe and intermittent or chronic. These include autoimmune hemolytic anemia, autoimmune neutropenia, and autoimmune thrombocytopenia. Other autoimmune manifestations can be similar to systemic lupus erythematosus (least common, affecting <5% of patients). Manifestations within the nervous system can include autoimmune cerebellar ataxia, Guillain–Barré syndrome, and transverse myelitis. Manifestations in the gastrointestinal system can include atrophic gastritis, and autoimmune hepatitis, esophagitis, colitis, and pancreatitis.
Pinworm infection (threadworm infection in the UK), also known as enterobiasis, is a human parasitic disease caused by the pinworm. The most common symptom is itching in the anal area. This can make sleeping difficult. The period of time from swallowing eggs to the appearance of new eggs around the anus is 4 to 8 weeks. Some people who are infected do not have symptoms.The disease is spread between people by pinworm eggs. The eggs initially occur around the anus and can survive for up to three weeks in the environment. They may be swallowed following contamination of the hands, food, or other articles. Those at risk are those who go to school, live in a health care institution or prison, or take care of people who are infected. Other animals do not spread the disease. Diagnosis is by seeing the worms which are about one centimetre long or the eggs under a microscope.Treatment is typically with two doses of the medications mebendazole, pyrantel pamoate, or albendazole two weeks apart. Everyone who lives with or takes care of an infected person should be treated at the same time. Washing personal items in hot water after each dose of medication is recommended. Good handwashing, daily bathing in the morning, and daily changing of underwear can help prevent reinfection.Pinworm infections commonly occur in all parts of the world. They are the most common type of worm infection in Western Europe and the United States. School-aged children are the most commonly infected.