Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue (D76) ICD-10-CM
The D76 code range covers other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue with 4 ICD-10-CM diagnosis codes. 3 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- Abt- Letterer-Siwe disease C96.0
- eosinophilic granuloma C96.6
- Hand-Schüller-Christian disease C96.5
- histiocytic medullary reticulosis C96.9
- histiocytic sarcoma C96.A
- histiocytosis X, multifocal C96.5
- histiocytosis X, unifocal C96.6
- Langerhans-cell histiocytosis, multifocal C96.5
- Langerhans-cell histiocytosis NOS C96.6
- Langerhans-cell histiocytosis, unifocal C96.6
- leukemic reticuloendotheliosis C91.4
- lipomelanotic reticulosis I89.8
- malignant histiocytosis C96.A
- malignant reticulosis C86.0
- nonlipid reticuloendotheliosis C96.0
Codes in the D76 Range 4 codes · 3 billable
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the D76 range.
Macrophage Activation Syndrome
A serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T-LYMPHOCYTES and MACROPHAGES. It is seen predominantly in children with systemic onset JUVENILE IDIOPATHIC ARTHRITIS.
Necrobiotic Xanthogranuloma
A cutaneous necrobiotic disorder characterized by firm, yellow plaques or nodules, often in a periorbital distribution. It is often accompanied by an elevated ERYTHROCYTE SEDIMENTATION RATE; LEUKOPENIA; and MONOCLONAL GAMMOPATHY (IgG-kappa type) and systemic involvement.
About the D76 Code Range
ICD-10 code D76 covers a group of rare disorders involving the lymphoreticular and reticulohistiocytic tissues, primarily affecting certain immune cells. These conditions include specific types of hemophagocytic syndromes and histiocytosis syndromes, each with distinct causes and clinical features.
Code D76.1 is designated for hemophagocytic lymphohistiocytosis (HLH), a severe immune activation disorder known under various names such as macrophage activation syndrome and familial hemophagocytic lymphohistiocytosis. The ICD-10 code for HLH helps identify cases where this life-threatening condition requires precise diagnosis and treatment. D76.2 is used when the hemophagocytic syndrome is triggered by an infection, sometimes called infection-associated macrophage activation syndrome. For other unusual histiocytic disorders, D76.3 captures diverse conditions like juvenile xanthogranuloma and Rosai-Dorfman disease, encompassing both benign and progressive forms. These ICD-10 codes assist healthcare providers and coders in reporting and managing complex diseases affecting white blood cells and related tissue systems.
Questions About This Page
How many billable codes are in the D76 range?
Of the 4 codes in this range, 3 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the D76 range classify?
The range classifies other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
