AutoICD API

D76.1

Billable

Hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis

Status

Billable / Specific

Block

D70-D77

Parent Code

D76

ICD-11 Mapping

1 equivalent

Coding Notes

Inclusion Terms

Alternative clinical terms for this condition

  • Familial hemophagocytic reticulosis
  • Histiocytoses of mononuclear phagocytes

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(2)
D76.2

Hemophagocytic syndrome, infection-associated

BillableD76.3

Other histiocytosis syndromes

Billable
ICD-11 Equivalents(1)

ICD-11 Equivalents

View full mapping

Corresponding ICD-11 codes from the WHO crosswalk mapping

4A01.23Primary haemophagocytic lymphohistiocytosisequivalentPrimary
Also Known As / Clinical Terms(227)

SNOMED CT

UMLS

Clinical Terms

  • Neonatal anemia
  • Familial Hemophagocytic Lymphocytoses
  • Primary haemophagocytic lymphohistiocytosis
  • Lymphohistiocytosis, Familial Erythrophagocytic
  • Primary Hemophagocytic Hymphohistiocytosis
  • Secondary haemophagocytic lymphohistiocytosis
  • Hemophagocytic Hymphohistiocytoses, Primary
  • Primary hemophagocytic lymphohistiocytosis
  • Erythrophagocytic Lymphohistiocytosis, Familial
  • familial hemophagocytic lymphohistiocytosis
  • Histiocytoses, Familial Hemophagocytic
  • Familial Hemophagocytic Histiocytoses
  • Hemophagocytic Hymphohistiocytosis, Primary
  • Lymphohistiocytoses, Familial Hemophagocytic
  • Familial Hemophagocytic Histiocytosis
  • Disorder of haematopoietic system in newborn
  • Genetic hemophagocytic lymphohistiocytosis
  • Hemophagocytic Reticulosis, Familial
  • Genetic haemophagocytic lymphohistiocytosis
  • Familial Hemophagocytic Lymphocytosis
  • FHLH
  • Erythrophagocytic Lymphohistiocytoses, Familial
  • Familial hemophagocytic reticulosis
  • Hemophagocytic Lymphohistiocytoses, Primary
  • NOCARH syndrome
  • Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
  • Familial Histiocytic Reticulosis
  • Hemophagocytic Syndrome
  • Secondary hemophagocytic lymphohistiocytosis
  • Macrophage activation syndrome
  • Familial Histiocytic Reticuloses
  • Hemophagocytic Lymphocytosis, Familial
  • Hyperinflammatory lymphohistiocytosis
  • Reticuloses, Familial Histiocytic
  • Haemophagocytic lymphohistiocytosis with rheumatologic disease
  • Histiocytic Reticuloses, Familial
  • Hemophagocytic Lymphohistiocytoses, Familial
  • Haemophagocytic lymphohistiocytosis
  • Histiocytic Reticulosis, Familial
  • Malignant white blood cell disorder
  • Hemophagocytic lymphohistiocytosis (disorder)
  • Hemophagocytic Lymphohistiocytosis, Primary
  • Disorder of hematopoietic system in newborn
  • Neonatal purpura
  • Neonatal anaemia
  • Lymphocytosis, Familial Hemophagocytic
  • Hemophagocytic Reticuloses, Familial
  • Lymphohistiocytosis, Familial Hemophagocytic
  • Acquired haemophagocytic lymphohistiocytosis associated with malignant disease
  • Reactive hemophagocytic syndrome
  • FHL - Familial haemophagocytic lymphohistiocytosis
  • Hemophagocytic Histiocytoses, Familial
  • Neonatal onset cytopenia, autoinflammation, rash, episodes of hemophagocytic lymphohistiocytosis syndrome
  • Familial Erythrophagocytic Lymphohistiocytosis
  • Hemophagocytic Lymphocytoses, Familial
  • Familial haemophagocytic lymphohistiocytosis
  • Hemophagocytic Disorder
  • Hymphohistiocytoses, Primary Hemophagocytic
  • Histiocytosis, Familial Hemophagocytic
  • Primary Hemophagocytic Hymphohistiocytoses
  • Lymphohistiocytoses, Familial Erythrophagocytic
  • Reticulosis, Familial Histiocytic
  • Familial Hemophagocytic Reticuloses
  • Hemophagocytic Lymphohistiocytoses
  • Familial haemophagocytic histiocytosis
  • Lymphohistiocytosis, Primary Hemophagocytic
  • Acquired pancytopenia
  • Familial Erythrophagocytic Lymphohistiocytoses
  • Primary Hemophagocytic Lymphohistiocytoses
  • Macrophage activation syndrome due to juvenile systemic onset arthritis
  • Reactive haemophagocytic syndrome
  • FHL - Familial hemophagocytic lymphohistiocytosis
  • Familial haemophagocytic reticulosis
  • Haemophagocytic syndrome
  • Reticulosis, Familial Hemophagocytic
  • HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS, FAMILIAL
  • HPLH
  • Lipochrome histiocytosis - familial
  • Histiocytoses of mononuclear phagocytes
  • Neonatal thrombocytopenia
  • Hymphohistiocytosis, Primary Hemophagocytic
  • Reticuloses, Familial Hemophagocytic
  • FHL
  • Pancytopenia - acquired
  • Lymphohistiocytosis, Hemophagocytic
  • FEL
  • Familial hemophagocytic lymphohistiocytosis (disorder)
  • Lymphocytoses, Familial Hemophagocytic
  • Hemophagocytic Histiocytosis, Familial
  • Lymphohistiocytoses, Hemophagocytic
  • Hemophagocytic lymphohistiocytosis with rheumatologic disease
  • Purpura of newborn
  • Lymphohistiocytoses, Primary Hemophagocytic
  • Familial Hemophagocytic Lymphohistiocytoses
  • FEL - Familial erythrophagocytic lymphohistiocytosis
Frequently Asked Questions
What is the ICD-10 code for hemophagocytic lymphohistiocytosis?

The ICD-10-CM code for hemophagocytic lymphohistiocytosis is D76.1. The full clinical description is "Hemophagocytic lymphohistiocytosis". D76.1 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code D76.1 mean?

ICD-10-CM code D76.1 represents “Hemophagocytic lymphohistiocytosis”. It is classified under Chapter 3: Diseases of the Blood and Blood-Forming Organs and is a billable/specific code that can be used on a claim.

Is D76.1 a billable code?

Yes, D76.1 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is D76.1 in?

D76.1 is in Chapter 3: Diseases of the Blood and Blood-Forming Organs (codes D50-D89).

What codes cannot be used with D76.1?

D76.1 has Excludes1 notes indicating codes that cannot be used together with it, including: (Abt-) Letterer-Siwe disease (C96.0); eosinophilic granuloma (C96.6); Hand-Schüller-Christian disease (C96.5); and 12 more.

What SNOMED CT codes does D76.1 map to?

D76.1 maps to 13 SNOMED CT concepts: 782915004, 5876000, 414028007, 398250003, 234437005, and 8 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for D76.1?

D76.1 is linked to 3 UMLS Concept Unique Identifiers: C0272199, C0024291, C1399675. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does D76.1 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like hemophagocytic lymphohistiocytosis affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of D76.1?

D76.1 maps to the ICD-11 code: 4A01.23 (Primary haemophagocytic lymphohistiocytosis).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.