AHA Coding Clinic® for ICD-9 - 2009 Issue 4; VOLUMES 1 & 2 NEW/REVISED CODES

Autoimmune Lymphoproliferative Syndrome

Effective October 1, 2009, code 279.4, Autoimmune disease, not elsewhere classified, has been expanded to uniquely identify autoimmune lymphoproliferative syndrome (ALPS). Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disorder of the immune system. It can affect both children and adults. In ALPS, unusually high numbers of lymphocytes accumulate in the lymph nodes, liver, and spleen, which can lead to enlargement of these organs. ALPS can cause numerous autoimmune problems. These problems include anemia, thrombocytopenia, and neutropenia. There is no cure for ALPS. However, most of its complications can be treated with splenectomy, steroids, or blood transfusions. 279 Disorders...

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