D76.1 – Hemophagocytic lymphohistiocytosis

ICD-10-CM 2027 diagnosis code · Other disorders of blood and blood-forming organs

Billable code

ICD-10-CM code D76.1
Code
D76.1 (claims format: D761)
Description
Hemophagocytic lymphohistiocytosis
Billable
Yes – valid for HIPAA-covered transactions
Valid for
Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter
3. Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
Block
D70-D77 Other disorders of blood and blood-forming organs
Parent codes
D76

Notes for D76.1

Applicable to:
  • Familial hemophagocytic reticulosis
  • Histiocytoses of mononuclear phagocytes

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

D76 – Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

Excludes1 (not coded here):
  • (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)

Index terms for D76.1

Entries in the ICD-10-CM alphabetic index that lead to this code:

Related codes in D76

Frequently asked questions

What is ICD-10 code D76.1?

D76.1 is the ICD-10-CM code for hemophagocytic lymphohistiocytosis, in the block D70-D77 (Other disorders of blood and blood-forming organs).

Is D76.1 a billable code?

Yes. D76.1 is a billable/specific code that can be used to indicate a diagnosis for reimbursement purposes.

What category does D76.1 belong to?

It belongs to category D76 – Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.