D76.1 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

Code Information

Diagnosis Code: D76.1
Short Description: Hemophagocytic lymphohistiocytosis
Long Description: Hemophagocytic lymphohistiocytosis
Code Version: 2025 ICD-10-CM

The code D76.1 is VALID for claim submission

Code Classification

Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
- Other disorders of blood and blood-forming organs (D70-D77)
  - Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue (D76)

Synonyms

Acquired hemophagocytic lymphohistiocytosis associated with malignant disease
Familial hemophagocytic lymphohistiocytosis
Hemolytic erythrophagocytic syndrome
Hemolytic erythrophagocytic syndrome
Hemolytic erythrophagocytic syndrome
Hemolytic erythrophagocytic syndrome
Hemolytic erythrophagocytic syndrome
Hemolytic erythrophagocytic syndrome
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis
Lipochrome histiocytosis - familial
Macrophage activation syndrome
Macrophage activation syndrome
Macrophage activation syndrome due to juvenile systemic onset arthritis
Malignant white blood cell disorder
Secondary hemophagocytic lymphohistiocytosis
Secondary hemophagocytic lymphohistiocytosis

Diagnostic Related Group(s)

The code D76.1 is grouped in the following Diagnostic Related Group(s) MS-DRG V42.0

Reticuloendothelial And Immunity Disorders With Mcc (814)
Reticuloendothelial And Immunity Disorders With Cc (815)
Reticuloendothelial And Immunity Disorders Without Cc/mcc (816)

References to Index of Diseases and Injuries

The code D76.1 has the following ICD-10-CM references to the Index of Diseases and Injuries

Inclusion Terms:
- Familial hemophagocytic reticulosis
- Histiocytoses of mononuclear phagocytes

Crosswalk Information

ICD-10 Code		ICD-9 Code	ICD-9 Description
D76.1		288.4	Hemophagocytic syndromes

This ICD-10 to ICD-9 data is based on the 2018 General Equivalency Mapping (GEM) files published by the Centers for Medicare & Medicaid Services (CMS) for informational purposes only. The data is not an ICD-10 conversion tool and doesn’t guarantee clinical accuracy.

Similar Codes

ICD-10 Code	ICD-10 Description
D76	Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
D76.2	Hemophagocytic syndrome, infection-associated
D76.3	Other histiocytosis syndromes

This page was last updated on: 10/1/2024

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