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D76.1

Hemophagocytic lymphohistiocytosis

Billable FY2026 2025-10-01 → 2026-09-30

Exclusions 24 never code together

D76code

Excludes1

(Abt-) Letterer-Siwe disease (C96.0)C96.0
eosinophilic granuloma (C96.6)C96.6
Hand-Schüller-Christian disease (C96.5)C96.5
histiocytic medullary reticulosis (C96.9)C96.9
histiocytic sarcoma (C96.A)C96.A
histiocytosis X, multifocal (C96.5)C96.5
histiocytosis X, unifocal (C96.6)C96.6
Langerhans-cell histiocytosis, multifocal (C96.5)C96.5
Langerhans-cell histiocytosis NOS (C96.6)C96.6
Langerhans-cell histiocytosis, unifocal (C96.6)C96.6
leukemic reticuloendotheliosis (C91.4-)C91.4-
lipomelanotic reticulosis (I89.8)I89.8
malignant histiocytosis (C96.A)C96.A
malignant reticulosis (C86.0)C86.0
nonlipid reticuloendotheliosis (C96.0)C96.0

D50-D89chapter

Excludes2

autoimmune disease (systemic) NOS (M35.9)M35.9
certain conditions originating in the perinatal period (P00-P96)P00-P96
complications of pregnancy, childbirth and the puerperium (O00-O9A)O00-O9A
congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)Q00-Q99
endocrine, nutritional and metabolic diseases (E00-E88)E00-E88
human immunodeficiency virus [HIV] disease (B20)B20
injury, poisoning and certain other consequences of external causes (S00-T88)S00-T88
neoplasms (C00-D49)C00-D49
symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)R00-R94

Excludes1 — never code together. The excluded condition and this one are mutually exclusive.

Excludes2 — not included here, but the patient may have both. Code both when documented.

Instructional notes 2

D76.1code

Inclusion terms

Familial hemophagocytic reticulosis
Histiocytoses of mononuclear phagocytes

Official Guidelines 1 names this code

ICD-10-CM Official Guidelines for Coding and Reporting, quoted in full. Published by CMS and NCHS; in the public domain.

I.C.3 Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) names this code p.39

Reserved for future guideline expansion

Index entries leading here 4

Reticulosis (skin)
- hemophagocytic, familial D76.1
Histiocytosis D76.3
- mononuclear phagocytes NEC D76.1
Lymphohistiocytosis, hemophagocytic (familial) D76.1
Syndrome — see also Disease
- macrophage activation D76.1

Alongside this code 2 same parent — D76

D76.2 Hemophagocytic syndrome, infection-associated D76.3 Other histiocytosis syndromes

Medications 1

2 Lymphohistiocytosis, Hemophagocytic drugs

MED-RT files conditions by MeSH, which carves the world differently from ICD-10. These are name matches on this code's description, offered as candidates — not as an assertion that they are this code.

Additional references

AHA Coding Clinic® Licence required

Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.

Risk adjustment

CMS22HCC47 CMS28HCC115 ESRD21HCC47 ESRD24HCC47

Presence is a property of the code. Untick a model to hide it everywhere.

This code

Billable: yes
Code set: FY2026
Parent: D76
Block: D70-D77
Siblings: 2

Present FY2024–FY2027.