Autoimmune lymphoproliferative syndrome [ALPS] digital illustration

Autoimmune lymphoproliferative syndrome [ALPS] Save


ICD-10 code: D89.82

Chapter: Diseases of the blood and blood forming organs and certain disorders involving the immune mechanism

Autoimmune lymphoproliferative syndrome [ALPS]

Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder that affects the immune system. This condition is caused by mutations in genes that control the regulation of lymphocytes, which are a type of white blood cell that helps fight infections. People with ALPS have an overproduction of lymphocytes, leading to an enlarged spleen and lymph nodes. They are also at risk of developing autoimmune diseases and certain types of cancer.

The symptoms of ALPS can vary from person to person, but typically include chronic or recurrent infections, enlarged lymph nodes and spleen, autoimmune disorders such as rheumatoid arthritis and lupus, and an increased risk of certain types of cancer. Diagnosis of ALPS is made through a combination of clinical symptoms, blood tests, and genetic testing.

Treatment for ALPS typically involves managing the symptoms and complications of the condition. This may include antibiotics to treat infections, immunosuppressive drugs to control autoimmune symptoms, and surgery to remove an enlarged spleen. In some cases, bone marrow transplantation may be necessary.

  1. Antibiotics
  2. Immunosuppressive drugs
  3. Surgery
  4. Bone marrow transplantation

It is important for people with ALPS to receive ongoing medical care and monitoring, as they are at an increased risk of developing certain types of cancer. Regular screening for cancer is recommended, as well as genetic counseling for affected individuals and their families.

In conclusion, autoimmune lymphoproliferative syndrome is a rare genetic disorder that affects the immune system. It can cause an overproduction of lymphocytes, leading to an enlarged spleen and lymph nodes, as well as an increased risk of autoimmune diseases and certain types of cancer. Treatment typically involves managing symptoms and complications, and ongoing medical care and monitoring is important for those with this condition.

Diagnosis Codes for Autoimmune lymphoproliferative syndrome [ALPS] | D89.82