Autoimmune lymphoproliferative syndrome [ALPS]

This code identifies Autoimmune Lymphoproliferative Syndrome (ALPS), a rare genetic disorder characterized by defective lymphocyte apoptosis (programmed cell death). This defect leads to an accumulation of lymphocytes, resulting in chronic, non-malignant lymphadenopathy, splenomegaly, and autoimmune manifestations affecting various organs.

Assign this code for patients diagnosed with ALPS, typically confirmed through genetic testing and characteristic clinical findings. Documentation should clearly indicate the diagnosis of ALPS, often including details of chronic lymphadenopathy, splenomegaly, and autoimmune cytopenias. This code is appropriate when managing the long-term care and complications associated with this specific syndrome.

• Ensure documentation specifies "Autoimmune Lymphoproliferative Syndrome" to differentiate from other lymphoproliferative disorders.
• Code any associated autoimmune manifestations (e.g., autoimmune hemolytic anemia, immune thrombocytopenia) separately as secondary diagnoses.
• Do not confuse with other unspecified or non-autoimmune lymphoproliferative conditions.