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. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 39 closely related codes. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Diseases of white blood cells.

For Medicare Advantage risk adjustment, D76.3 maps to CMS-HCC Category 115 (Specified Immunodeficiencies and White Blood Cell Disorders) under the V28 model, adding a risk factor of about 0.565 for a community, non-dual, aged beneficiary in payment year 2026.

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

Medicare Risk Adjustment (HCC)Billing

D76.3 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2026.

CMS-HCC V28 Category (Payment Model)
HCC 115— Specified Immunodeficiencies and White Blood Cell Disorders
Payment HCC · PY 2026 one of 19 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+0.565
community, non-dual, aged · ranges 0.302–0.692 across segments
Hierarchy
Superseded by HCC 114
a more severe related category takes the payment when both are reported
Prior Model (CMS-HCC V24)
HCC 47
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
PACE (CMS-HCC V22): HCC 47 · ESRD (V21): HCC 47 · ESRD (V24): HCC 47
ESRD V21 weights: 0.097 dialysis, 0.549–0.688 functioning graft · ESRD V24 weights: 0.078 dialysis, 0.523–0.803 functioning graft
Part D (RxHCC)
Not mapped
D76.3 does not risk-adjust in the RxHCC prescription drug model

Source: CMS Payment Year 2026 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

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.

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

What is the ICD-10 code for other histiocytosis syndromes?

The ICD-10-CM code for other histiocytosis syndromes is D76.3 (sometimes written as D763). It is billable on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

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.

Is D76.3 a CC or MCC?

CMS lists D76.3 as a CC (complication or comorbidity) for FY 2026. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 39 closely related codes in its exclusion list.

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.

What HCC is D76.3?

D76.3 (other histiocytosis syndromes) maps to CMS-HCC Category 115 (Specified Immunodeficiencies and White Blood Cell Disorders), commonly written as HCC 115, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2026. It mapped to HCC 47 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. It does not map to any RxHCC in the Part D prescription drug model.

Does D76.3 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, D76.3 adds a risk adjustment factor of about 0.565 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.302 to 0.692 depending on the payment segment). A more severe related category (HCC 114) supersedes it when both are reported. See the full factor table on the HCC 115 category page.