E85.81
BillableLight chain (AL) amyloidosis
HCC Category Mapping
V28HCC 50 — Glycogen/Amino-Acid/Other Metabolic Disorders
0.289V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
A disease where abnormal light chain proteins accumulate in organs and tissues, causing damage and dysfunction. This is a type of amyloidosis that primarily affects the heart, kidneys, and nervous system.
Coding Tips
- •Specify the organ system affected when documented (cardiac, renal, or neurologic involvement)
- •Light chain amyloidosis is also known as AL amyloidosis; use this code when the diagnosis specifies light chain type
Clinical Significance
Light chain (AL) amyloidosis is the most common form of systemic amyloidosis, caused by misfolded immunoglobulin light chains from an underlying plasma cell dyscrasia. It commonly affects the heart, kidneys, liver, and nervous system, and carries significant morbidity and mortality. Accurate coding is critical for treatment planning with chemotherapy regimens.
Documentation Requirements
- ✓Confirmed AL amyloidosis diagnosis (tissue biopsy with immunohistochemistry or mass spectrometry showing light chain deposits)
- ✓Identification of underlying plasma cell disorder (monoclonal gammopathy, multiple myeloma)
- ✓Affected organ systems and staging (cardiac biomarkers, proteinuria, NT-proBNP, troponin)
- ✓Free light chain assay results (kappa/lambda ratio)
- ✓Current treatment regimen (bortezomib-based, daratumumab, stem cell transplant evaluation)
- ✓Organ response and hematologic response assessment
Commonly Confused Codes
E85.82 — Wild-type transthyretin-related (ATTR) amyloidosis: different protein (transthyretin, not light chains)E85.3 — Secondary systemic amyloidosis: reactive AA amyloidosis from chronic inflammation, not plasma cell diseaseC90.00 — Multiple myeloma: may be the underlying cause; code both when applicableE85.1 — Neuropathic heredofamilial amyloidosis: hereditary form, not acquired
Code Hierarchy
└E85Amyloidosis└E85.8Other amyloidosis└E85.81Light chain (AL) amyloidosis
└E85.81Light chain (AL) amyloidosis