Signs and symptoms of ALPS

ALPS shows up in various ways at different stages of life. Lymphoproliferation affecting the spleen and lymph nodes generally appears during childhood. Parents may notice persistently enlarged glands in the child’s neck, armpits and groin. Often there are no other symptoms at this stage.

Patients with ALPS are at increased risk of autoimmune disease, which occurs when the immune system malfunctions and attacks the body’s own tissues. A classic form of ALPS-related autoimmunity occurs when antibodies are formed that attack red blood cells (haemolytic anaemia) and platelets (thrombocytopenia), leading to tiredness and pallor, easy bruising and bleeding. This combination is called Evans syndrome. Other autoimmune diseases are much less common.

Although the majority of people with ALPS do not get lymphoma, ALPS is associated with an increased risk of this cancer of the white blood cells in adulthood. Lymphoma causes symptoms such as fever, fatigue, loss of appetite, weight loss or more marked enlargement of one or more lymph nodes. Doctors and patients will look out for these features so that appropriate tests and
treatment can be arranged.