D76.1 Hemophagocytic lymphohistiocytosis

The ICD-10-CM code for Hemophagocytic lymphohistiocytosis is D76.1 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

3: Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

Section

D70-D77: Other disorders of blood and blood-forming organs (D70-D77)

Category D76

4 codes (3 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to D76.1:

• Lymphohistiocytosis, hemophagocytic (familial)
• Reticulosis (skin) › hemophagocytic, familial
• Syndrome › macrophage activation
• Histiocytosis › mononuclear phagocytes NEC

Inclusion Terms

• Familial hemophagocytic reticulosis
• Histiocytoses of mononuclear phagocytes

U.S. Hospital Utilization

• An estimated 4,615 U.S. inpatient stays in 2023 included D76.1 among the documented diagnoses.
• 1,025 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Chapter 3 TOC entry

3. Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) ............................................................................................. 39

Reserved for future guideline expansion

Reserved for future guideline expansion ...................................................................................... 39

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)