D89.82
BillableAutoimmune lymphoproliferative syndrome [ALPS]
HCC Category Mapping
V24HCC 47 — Disorders of Immunity
0.472ESRDHCC 47 — Disorders of Immunity
0.000What This Code Means
A rare inherited immune disorder where the body cannot properly control lymphocyte (immune cell) growth, leading to enlarged lymph nodes, spleen, and increased risk of infections and cancers.
Coding Tips
- •ALPS is a genetic disorder; document any family history and genetic testing results in the medical record
- •This is a rare disease; ensure proper documentation to support medical necessity for specialized treatments
Clinical Significance
Autoimmune lymphoproliferative syndrome is a rare inherited disorder of lymphocyte apoptosis, typically caused by mutations in the FAS gene, leading to chronic lymphoproliferation, autoimmune cytopenias (particularly autoimmune hemolytic anemia and immune thrombocytopenia), and increased risk of lymphoma. It typically presents in childhood with splenomegaly and lymphadenopathy. Lifelong monitoring for malignant transformation is essential.
Documentation Requirements
- ✓Documentation should include the confirmed diagnosis of autoimmune lymphoproliferative syndrome with supporting evidence such as elevated double-negative T cells (CD3+CD4-CD8-), FAS gene mutation analysis, elevated biomarkers (soluble FAS ligand, interleukin-10, vitamin B12), and clinical features.
- ✓Current treatment (immunosuppressive agents, sirolimus, mycophenolate), presence of autoimmune cytopenias, and lymphoma surveillance status should be documented.
Commonly Confused Codes
D47.Z1 (Post-transplant lymphoproliferative disorder) involves lymphoproliferation after transplant immunosuppression, not a genetic defect. C85.9(Non-Hodgkin lymphoma, unspecified) should be coded if malignant transformation has occurred. D89.89 (Other specified disorders involving the immune mechanism) is less specific and should not be used when autoimmune lymphoproliferative syndrome is confirmed. D69.3 (Immune thrombocytopenic purpura) may be an associated finding but does not replace this code.