Conditions / Immune
autoimmune lymphoproliferative syndrome
info · Immune · ICD-10: D89.82
A hypersensitivity reaction type IV disease that is an inherited disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). It is characterized by the production of an abnormally large number of lymphocytes. Accumulati
A hypersensitivity reaction type IV disease that is an inherited disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). It is characterized by the production of an abnormally large number of lymphocytes. Accumulation of excess lymphocytes results in enlargement of the lymph nodes, the liver, and the spleen.
Signs and symptoms
- Iron deficiency anemia
- Antineutrophil antibody positivity
- Platelet antibody positive
- Increased circulating IgM concentration
- Autoimmune hemolytic anemia
- Antinuclear antibody positivity
- Hepatomegaly
- Autoimmune neutropenia
- Splenomegaly
- Decreased T cell apoptosis
Also known as: ALPS; Canale-Smith syndrome