An inherited disorder of the immune system that affects both children and adults.
Unusually high numbers of white blood cells accumulate in the lymph nodes, liver,
and spleen, which can lead to enlargement of these organs.
ALPS can cause numerous autoimmune problems such as:
Anaemia (low count of red blood cells).
Thrombocytopenia (low count of platelets).
Neutropenia (low count of neutrophils, the most common type of white blood cell).
Tends to affect both men and women equally.
Splenectomy
An enlarged spleen is common in people with ALPS. Usually it is not necessary to
remove the spleen unless there are problems encountered such as:
Anaemia.
Thrombocytopenia.
Concern that the spleen may rupture due to massive enlargement.
Removing a spleen carries both risks and benefits.
Benefits, such as:
Easier to regulate and control blood counts
Less discomfort
No risk of spleen rupture
Risks, such as:
Increased likelihood of certain bacterial infections, which can be life threatening;
people must get vaccinated to avoid infections, and some may need to take antibiotics
for many years
Possible recurrence of anaemia or thrombocytopenia.
Some signs of Autoimmune Lymphoproliferative Syndrome (ALPS) are ones people can
feel or see, while other signs can be detected only by laboratory tests, such as:
An enlarged spleen.
Enlarged lymph nodes, especially in the neck and underarms.
An enlarged liver.
Skin rashes.
Frequent nose bleeds.
Anaemia (low blood counts).
An increase in certain types of white blood cells (including double-negative T cells).
An increased life-span of some white blood cells that are no longer needed.
