D76.3
Other histiocytosis syndromes
D76.3 (also written D763) is a billable ICD-10-CM diagnosis code for other histiocytosis syndromes. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 3 codes in category D76 (Other specified diseases with participation of lymphoreticular and reticulohistiocytic tissue). The Alphabetic Index lists it under 12 entries. Its coding notes include 15 Excludes1 entries and 9 Excludes2 entries.
Excludes1
Not coded here. The listed conditions generally should not be reported together.
- (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)
Excludes2
Not included here. Both codes may be reported if the patient has both conditions.
Also from chapter/block level (9 notes)
- autoimmune disease (systemic) NOS (M35.9)
- certain conditions originating in the perinatal period (P00-P96)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- human immunodeficiency virus [HIV] disease (B20)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Care settings
How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.
Home Health Primary
Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the MMTA - Infectious Disease, Neoplasms, and Blood-Forming Diseases clinical group.
Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026Hospital CC
Counts as a complication or comorbidity (CC) when reported as a secondary diagnosis on an inpatient claim. It does not count when the principal diagnosis is one of the 39 codes in its exclusion list. Some MS-DRGs also ignore a CC or MCC that defines the DRG.
Source: CMS MS-DRG Definitions Manual v44, effective October 1, 2026, Appendix CInclusion terms
Conditions classified to this code.
- Reticulohistiocytoma (giant-cell)
- Sinus histiocytosis with massive lymphadenopathy
- Xanthogranuloma
Alphabetic Index terms
Terms the official Alphabetic Index lists for this code. 12 entries
- 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