ऑटोइम्यून लिम्फोप्रोलिफेरेटिव सिंड्रोम
अंतिम अद्यतन 18 Aug 2020
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Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). ALPS is characterised by the production of an abnormally large number of lymphocytes (lymphoproliferation). Accumulation of excess lymphocytes results in enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly), and the spleen (splenomegaly).
विवरण
Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). ALPS is characterised by the production of an abnormally large number of lymphocytes (lymphoproliferation). Accumulation of excess lymphocytes results in enlargement of the lymph nodes (lymphadenopathy), the liver (hepatomegaly), and the spleen (splenomegaly).
Autoimmune disorders are also common in ALPS. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. Most of the autoimmune disorders associated with ALPS target and damage blood cells. For example, the immune system may attack red blood cells (autoimmune hemolytic anaemia), white blood cells (autoimmune neutropenia), or platelets (autoimmune thrombocytopenia). Less commonly, autoimmune disorders that affect other organs and tissues occur in people with ALPS. These disorders can damage the kidneys (glomerulonephritis), liver (autoimmune hepatitis), eyes (uveitis), or nerves (Guillain-Barre syndrome). Skin problems, usually rashes or hives (urticaria), can also occur in ALPS.
ALPS can have varying patterns of signs and symptoms. Most commonly, lymphoproliferation becomes apparent during childhood. Enlargement of the lymph nodes and spleen frequently occur in affected individuals. Autoimmune disorders typically develop several years later, most frequently as a combination of hemolytic anaemia and thrombocytopenia, also called Evans syndrome. People with this classic form of ALPS generally have a near-normal lifespan, but have a greatly increased risk of developing cancer of the immune system cells (lymphoma) compared with the general population.
Some people have signs and symptoms that resemble those of ALPS, including lymphoproliferation, lymphadenopathy, splenomegaly, and low blood counts, but the specific pattern of these signs and symptoms or the genetic cause may be different. Researchers disagree whether individuals with these non-classic forms should be considered to have ALPS or a separate condition.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant; Autosomal recessive. More than one pattern can apply in different cases.
Related genes
Other names
ALPS
Canale-Smith syndrome
Related patient information and medicines
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स्जोग्रेन सिंड्रोम
Sjögren's syndrome is an autoimmune disease that causes the glands that produce tears and saliva to stop working. It is a life-long condition but treatments can help control symptoms such as dry eyes and dry mouth. What is Sjögren's syndrome. It can also affect other organs including the lungs, kidneys, skin and the nervous system. Sjögren's.
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नेफ्रोटिक सिंड्रोम
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Patient information leaflet
गुइलेन-बैरे सिंड्रोम
Guillain-Barré syndrome affects the nerves of the limbs and body and is usually triggered by an infection. What is Guillain-Barré syndrome. गुइलेन-बैरे सिंड्रोम (GBS) है a disease that affects nerves throughout the body and limbs. It is usually triggered by an infection. GBS has also been reported after certain vaccinations, but this is very.
Patient information leaflet
ऑटोइम्यून हेपेटाइटिस
ऑटोइम्यून हेपेटाइटिस is an uncommon cause of persistent liver inflammation (chronic hepatitis). The cause is not known. If left untreated, the inflammation causes 'scarring' of the liver (cirrhosis). However, with treatment, the outlook for people with this condition is very good. Treatment is usually with steroids and other medicines which.
Patient information leaflet
एंटीफॉस्फोलिपिड सिंड्रोम
एंटीफॉस्फोलिपिड सिंड्रोम is sometimes called Hughes' syndrome. It is a disease that affects the blood and makes it more likely to clot than normal - a thrombophilia. Antiphospholipid syndrome (APS) - also called Hughes' syndrome - makes blood more likely than normal to clot (a thrombophilia ). This increases the risk of developing blood clo....
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क्लाइनफेल्टर सिंड्रोम
क्लाइनफेल्टर सिंड्रोम is a genetic condition that only affects males. Affected males have an extra X chromosome. It is sometimes just called XXY. Between 1 in 500 and 1 in 1,000 boys are born with Klinefelter's syndrome (KS). Many people with KS are not diagnosed until they are adults, and it is believed that up to two thirds may never be.
अधिक पठन और संदर्भ
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