ICD-10 Code D89.82 – Autoimmune lymphoproliferative syndrome [ALPS] (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D89.82 – Autoimmune lymphoproliferative syndrome [ALPS]
What it is
D89.82 identifies autoimmune lymphoproliferative syndrome, a rare disorder where the immune system does not properly regulate lymphocyte survival. This can lead to enlarged lymph nodes, spleen, or liver and autoimmune complications.
Clinical signs
Typical findings include chronic, nonmalignant lymphadenopathy, splenomegaly, and autoimmune cytopenias. Clinical features vary; refer to documentation for the specific manifestations supporting ALPS.
When to use this code
Use D89.82 when the record clearly documents ALPS or autoimmune lymphoproliferative syndrome as the diagnosis. It is appropriate for encounters focused on this confirmed condition, including evaluation of persistent lymphoid enlargement or autoimmune complications linked to ALPS.
Do not use for
Do not use this code for nonspecific lymphadenopathy, splenomegaly, or autoimmune disease without documented ALPS. Check documentation if the note only suggests an immune disorder or lymphoproliferative process.
Coding tip
Assign D89.82 only when the provider names ALPS explicitly; otherwise, code the documented signs or confirmed alternate diagnosis.