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  2. ICD-10-CM Codes List
  3. D50–D89
  4. D70-D77
  5. Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue (D76)

Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue (D76)

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.

Instructional Notations

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

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

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.