4A01.23
Primary haemophagocytic lymphohistiocytosis
Primary haemophagocytic lymphohistiocytosis
Classification
ICD-11
Block
4A00-4A0ZParent Code
4A01.2ICD-10 Mapping
1 equivalentWHO Foundation
View on WHOICD-10 Equivalents(1)
ICD-10 Equivalents
View full mappingCorresponding ICD-10-CM codes from the WHO crosswalk mapping
Also Known As / Clinical Terms(12)
SNOMED CT ↗
- Acquired pancytopenia5876000
- Haemophagocytic lymphohistiocytosis234437005
- Lipochrome histiocytosis - familial234577004
- Malignant white blood cell disorder277543005
- FEL - Familial erythrophagocytic lymphohistiocytosis398250003
- Disorder of haematopoietic system in newborn414028007
- Haemophagocytic lymphohistiocytosis with rheumatologic disease430478003
- Acquired haemophagocytic lymphohistiocytosis associated with malignant disease782915004
- Macrophage activation syndrome due to juvenile systemic onset arthritis1156801007
- NOCARH syndrome1360083000
Frequently Asked Questions
What is the ICD-11 code for primary haemophagocytic lymphohistiocytosis?
The ICD-11 code for primary haemophagocytic lymphohistiocytosis is 4A01.23. The full clinical description is "Primary haemophagocytic lymphohistiocytosis".
What does ICD-11 code 4A01.23 mean?
ICD-11 code 4A01.23 represents “Primary haemophagocytic lymphohistiocytosis”. It is classified under Chapter 4: Diseases of the Immune System.
What chapter is 4A01.23 in?
4A01.23 is in Chapter 4: Diseases of the Immune System (codes 4A00-4B4Z).
What is the ICD-10 equivalent of ICD-11 code 4A01.23?
4A01.23 maps to the ICD-10 code: D76.1 (Hemophagocytic lymphohistiocytosis). This is an equivalent mapping.
What is the difference between ICD-10 and ICD-11?
ICD-11 is the latest revision of the WHO's International Classification of Diseases, succeeding ICD-10. Key differences include: a fully digital-first design, new chapters for sleep-wake disorders, sexual health, and traditional medicine, improved coding for rare diseases, and better integration with electronic health records through extension codes.
What SNOMED CT codes does 4A01.23 map to?
4A01.23 maps to 10 SNOMED CT concepts: 782915004, 5876000, 414028007, 398250003, 234437005, and 5 more. SNOMED CT is a clinical terminology used in electronic health records.
What UMLS concepts map to ICD-11 code 4A01.23?
4A01.23 is linked to 2 UMLS Concept Unique Identifiers: C0272199, 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.