Which histiocytosis code should you use?
The Alphabetic Index lists 8 codes under Histiocytosis. Which one applies depends on the qualifier documented in the record — the type, site, cause, or associated condition. Find the qualifier below, then verify the code in the Tabular List before using it.
By type
| acute differentiated progressive | C96.0 | Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis |
| Langerhans' cell NEC | C96.6 | Unifocal Langerhans-cell histiocytosis |
| lipid, lipoid | D76.3 | Other histiocytosis syndromes |
| malignant | C96.A | Histiocytic sarcoma |
| mononuclear phagocytes NEC | D76.1 | Hemophagocytic lymphohistiocytosis |
| non-Langerhans cell | D76.3 | Other histiocytosis syndromes |
| polyostotic sclerosing | D76.3 | Other histiocytosis syndromes |
| sinus, with massive lymphadenopathy | D76.3 | Other histiocytosis syndromes |
| syndrome NEC | D76.3 | Other histiocytosis syndromes |
| X NEC | C96.6 | Unifocal Langerhans-cell histiocytosis |
Histiocytosis Langerhans' cell NEC
| pulmonary, adult | J84.82 | Adult pulmonary Langerhans cell histiocytosis |
| unifocal | C96.6 | Unifocal Langerhans-cell histiocytosis |
| multifocal X › multisystemic | C96.0 | Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis |
| multifocal X › unisystemic | C96.5 | Multifocal and unisystemic Langerhans-cell histiocytosis |
Histiocytosis lipid, lipoid
| essential | E75.29 | Other sphingolipidosis |
Histiocytosis mononuclear phagocytes NEC
| Langerhans' cells | C96.6 | Unifocal Langerhans-cell histiocytosis |
Histiocytosis X NEC
| acute | C96.0 | Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis |
| chronic | C96.6 | Unifocal Langerhans-cell histiocytosis |
| multifocal | C96.5 | Multifocal and unisystemic Langerhans-cell histiocytosis |
| multisystemic | C96.0 | Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis |
| unifocal | C96.6 | Unifocal Langerhans-cell histiocytosis |