D76.1
BillableHemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis
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)
- (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)
Excludes 2
Conditions not included here, but the patient may have both
- autoimmune disease (systemic) NOS (M35.9)
- certain conditions originating in the perinatal period (P00-P96)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- human immunodeficiency virus [HIV] disease (B20)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Also Known As / Clinical Terms
SNOMED CT
- Acquired pancytopenia5876000
- Pancytopenia - acquired5876000
- Neonatal purpura82835005
- Neonatal thrombocytopenia82835005
- Purpura of newborn82835005
- Neonatal anaemia234350007
- Neonatal anemia234350007
- Haemophagocytic lymphohistiocytosis234437005
- Haemophagocytic syndrome234437005
- Hemophagocytic lymphohistiocytosis234437005
- Hemophagocytic syndrome234437005
- Hyperinflammatory lymphohistiocytosis234437005
- Lipochrome histiocytosis - familial234577004
- Malignant white blood cell disorder277543005
- FEL - Familial erythrophagocytic lymphohistiocytosis398250003
- FHL - Familial haemophagocytic lymphohistiocytosis398250003
- FHL - Familial hemophagocytic lymphohistiocytosis398250003
- Familial erythrophagocytic lymphohistiocytosis398250003
- Familial haemophagocytic histiocytosis398250003
- Familial haemophagocytic lymphohistiocytosis398250003
- Familial haemophagocytic reticulosis398250003
- Familial hemophagocytic histiocytosis398250003
- Familial hemophagocytic lymphohistiocytosis398250003
- Familial hemophagocytic reticulosis398250003
- Familial histiocytic reticulosis398250003
- Genetic haemophagocytic lymphohistiocytosis398250003
- Genetic hemophagocytic lymphohistiocytosis398250003
- Primary haemophagocytic lymphohistiocytosis398250003
- Primary hemophagocytic lymphohistiocytosis398250003
- Disorder of haematopoietic system in newborn414028007
- Disorder of hematopoietic system in newborn414028007
- Haemophagocytic lymphohistiocytosis with rheumatologic disease430478003
- Hemophagocytic lymphohistiocytosis with rheumatologic disease430478003
- Macrophage activation syndrome430478003
- Reactive haemophagocytic syndrome430478003
- Reactive hemophagocytic syndrome430478003
- Acquired haemophagocytic lymphohistiocytosis associated with malignant disease782915004
- Acquired hemophagocytic lymphohistiocytosis associated with malignant disease782915004
- Secondary haemophagocytic lymphohistiocytosis1153417000
- Secondary hemophagocytic lymphohistiocytosis1153417000
- Macrophage activation syndrome due to juvenile systemic onset arthritis1156801007
- NOCARH syndrome1360083000
- Neonatal onset cytopenia, autoinflammation, rash, episodes of hemophagocytic lymphohistiocytosis syndrome1360083000
UMLS
- ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS, FAMILIALC0272199
- Erythrophagocytic Lymphohistiocytoses, FamilialC0272199
- Erythrophagocytic Lymphohistiocytosis, FamilialC0272199
- FELC0272199
- FEL - Familial erythrophagocytic lymphohistiocytosisC0272199
- FHLC0272199
- FHL - Familial haemophagocytic lymphohistiocytosisC0272199
- FHL - Familial hemophagocytic lymphohistiocytosisC0272199
- FHLHC0272199
- Familial Erythrophagocytic LymphohistiocytosesC0272199
- Familial Erythrophagocytic LymphohistiocytosisC0272199
- Familial Hemophagocytic HistiocytosesC0272199
- Familial Hemophagocytic HistiocytosisC0272199
- Familial Hemophagocytic LymphocytosesC0272199
- Familial Hemophagocytic LymphocytosisC0272199
- Familial Hemophagocytic LymphohistiocytosesC0272199
- Familial Hemophagocytic LymphohistiocytosisC0272199
- Familial Hemophagocytic ReticulosesC0272199
- Familial Hemophagocytic ReticulosisC0272199
- Familial Histiocytic ReticulosesC0272199
- Familial Histiocytic ReticulosisC0272199
- Familial erythrophagocytic lymphohistiocytosisC0272199
- Familial haemophagocytic histiocytosisC0272199
- Familial haemophagocytic lymphohistiocytosisC0272199
- Familial haemophagocytic reticulosisC0272199
- Familial hemophagocytic histiocytosisC0272199
- Familial hemophagocytic lymphocytosisC0272199
- Familial hemophagocytic lymphohistiocytosisC0272199
- Familial hemophagocytic lymphohistiocytosis (disorder)C0272199
- Familial hemophagocytic reticulosisC0272199
- Familial histiocytic reticulosisC0272199
- Genetic haemophagocytic lymphohistiocytosisC0272199
- Genetic hemophagocytic lymphohistiocytosisC0272199
- HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS, FAMILIALC0272199
- HEMOPHAGOCYTIC RETICULOSIS, FAMILIALC0272199
- HPLHC0272199
- Hemophagocytic Histiocytoses, FamilialC0272199
- Hemophagocytic Histiocytosis, FamilialC0272199
- Hemophagocytic Hymphohistiocytoses, PrimaryC0272199
- Hemophagocytic Hymphohistiocytosis, PrimaryC0272199
- Hemophagocytic Lymphocytoses, FamilialC0272199
- Hemophagocytic Lymphocytosis, FamilialC0272199
- Hemophagocytic Lymphohistiocytoses, FamilialC0272199
- Hemophagocytic Lymphohistiocytoses, PrimaryC0272199
- Hemophagocytic Lymphohistiocytosis, FamilialC0272199
- Hemophagocytic Lymphohistiocytosis, PrimaryC0272199
- Hemophagocytic Reticuloses, FamilialC0272199
- Hemophagocytic Reticulosis, FamilialC0272199
- Hemophagocytic SyndromeC0272199
- Histiocytic Reticuloses, FamilialC0272199
- Histiocytic Reticulosis, FamilialC0272199
- Histiocytoses, Familial HemophagocyticC0272199
- Histiocytosis, Familial HemophagocyticC0272199
- Hymphohistiocytoses, Primary HemophagocyticC0272199
- Hymphohistiocytosis, Primary HemophagocyticC0272199
- Lymphocytoses, Familial HemophagocyticC0272199
- Lymphocytosis, Familial HemophagocyticC0272199
- Lymphohistiocytoses, Familial ErythrophagocyticC0272199
- Lymphohistiocytoses, Familial HemophagocyticC0272199
- Lymphohistiocytoses, Primary HemophagocyticC0272199
- Lymphohistiocytosis, Familial ErythrophagocyticC0272199
- Lymphohistiocytosis, Familial HemophagocyticC0272199
- Lymphohistiocytosis, Primary HemophagocyticC0272199
- Primary Hemophagocytic HymphohistiocytosesC0272199
- Primary Hemophagocytic HymphohistiocytosisC0272199
- Primary Hemophagocytic LymphohistiocytosesC0272199
- Primary Hemophagocytic LymphohistiocytosisC0272199
- Primary haemophagocytic lymphohistiocytosisC0272199
- Primary hemophagocytic hymphohistiocytosisC0272199
- Primary hemophagocytic lymphohistiocytosisC0272199
- RETICULOSIS, FAMILIAL HISTIOCYTICC0272199
- Reticuloses, Familial HemophagocyticC0272199
- Reticuloses, Familial HistiocyticC0272199
- Reticulosis, Familial HemophagocyticC0272199
- Reticulosis, Familial HistiocyticC0272199
- fHLHC0272199
- familial hemophagocytic lymphohistiocytosisC0272199
- Haemophagocytic lymphohistiocytosisC0024291
- Haemophagocytic syndromeC0024291
- Hemophagocytic DisorderC0024291
- Hemophagocytic LymphohistiocytosesC0024291
- Hemophagocytic LymphohistiocytosisC0024291
- Hemophagocytic lymphohistiocytosisC0024291
- Hemophagocytic lymphohistiocytosis (disorder)C0024291
- Hemophagocytic syndromeC0024291
- Hyperinflammatory lymphohistiocytosisC0024291
- Lymphohistiocytoses, HemophagocyticC0024291
- Lymphohistiocytosis, HemophagocyticC0024291
- Histiocytoses of mononuclear phagocytesC1399675
Frequently Asked Questions
What is ICD-10 code D76.1?
ICD-10-CM code D76.1 represents "Hemophagocytic lymphohistiocytosis". It 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.
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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.