2026 ICD-10-CM Diagnosis Code D76.3Other histiocytosis syndromes

ICD-10-CM Codes›D50–D89›D70-D77›D76

ICD-10-CM D76.3
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D76.3 is a billable ICD-10-CM diagnosis code for other histiocytosis syndromes. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 814 through 816. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Diseases of white blood cells.

Code Identity

ICD-10-CM Code
D76.3
Billable Status
Yes — Valid for Submission
Code Describes
Other histiocytosis syndromes
Short Description
Other histiocytosis syndromes
Same as the full description in the CMS dataset.
Parent Code
D76
Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD70-D77Other disorders of blood and blood-forming organs
CategoryD76Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue
This CodeD76.3Other histiocytosis syndromes

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Benign cephalic histiocytosis
  • Chronic histiocytosis
  • Cutaneous reticulohistiocytosis
  • Dermal dendritic cell histiocytosis
  • Erdheim-Chester disease
  • Hereditary progressive mucinous histiocytosis
  • Histiocytic syndrome
  • Histiocytosis-lymphadenopathy plus syndrome
  • Juvenile xanthogranuloma
  • Juvenile xanthogranuloma of iris
  • Juvenile xanthogranuloma of skin
  • Malakoplakia
  • Mucinous histiocytosis of the colon
  • Multiple eruptive juvenile xanthogranuloma
  • Necrobiotic xanthogranuloma
  • Necrobiotic xanthogranuloma with paraproteinemia
  • Non-Langerhans cell histiocytic dermatosis
  • Panniculitis secondary to histiocytic disorder
  • Periodontitis due to histiocytosis syndrome
  • Progressive nodular histiocytosis
  • Retroperitoneal xanthogranuloma
  • Rosai-Dorfman disease
  • Sea-blue histiocyte syndrome
  • Solitary eruptive xanthogranuloma
  • Solitary reticulohistiocytoma
  • Subcutaneous xanthogranulomatosis
  • Undetermined cell histiocytosis
  • Xanthogranuloma
  • Xanthogranuloma of choroid plexus

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Reticulohistiocytoma (giant-cell)
  • Sinus histiocytosis with massive lymphadenopathy
  • Xanthogranuloma

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Granuloma
      • reticulohistiocytic
    • Histiocytosis
    • Histiocytosis
      • lipid, lipoid
    • Histiocytosis
      • non-Langerhans cell
    • Histiocytosis
      • polyostotic sclerosing
    • Histiocytosis
      • sinus, with massive lymphadenopathy
    • Histiocytosis
      • syndrome NEC
    • Lipoid
      • histiocytosis
    • Reticulohistiocytoma(giant-cell)
    • Syndrome
      • histiocytic
    • Syndrome
      • histiocytosis NEC
    • Xanthogranuloma

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD007
Diseases of white blood cells
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Necrobiotic Xanthogranuloma

    a cutaneous necrobiotic disorder characterized by firm, yellow plaques or nodules, often in a periorbital distribution. it is often accompanied by an elevated erythrocyte sedimentation rate; leukopenia; and monoclonal gammopathy (igg-kappa type) and systemic involvement.

  • Malakoplakia

    an inflammatory reaction characterized by the presence of papules or nodules usually in the genitourinary tract. it is usually a reaction to an infection. morphologically, it consists of foamy histiocytes and characteristic basophilic inclusion bodies called michaelis-gutmann bodies.

Convert D76.3 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
288.4 Hemophagocytic syndromes
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About D76.3Overview

Is D76.3 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other histiocytosis syndromes on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does D76.3 group to?

When other histiocytosis syndromes is the principal diagnosis on an inpatient stay, it groups to MS-DRG 814, 815, 816, with relative weights from 0.6320 to 2.1267 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D76.3?

Under the General Equivalence Mappings, other histiocytosis syndromes converts to ICD-9-CM 288.4 (hemophagocytic syndromes). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.