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ICD-10-CM 2027

ICD-10 code for histiocytosis

D76.3

Other histiocytosis syndromes

✓ Billable / specificCC — complication/comorbidity

From the official ICD-10-CM alphabetic index entry “Histiocytosis”. Page updated September 29, 2026.

About coding histiocytosis

The ICD-10-CM code for histiocytosis is D76.3 (Other histiocytosis syndromes). The official index lists 21 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on D76.3.

Within D76, choose D76.3 (Other histiocytosis syndromes) only when documentation doesn't support a more specific option: Hemophagocytic lymphohistiocytosis (D76.1) and Hemophagocytic syndrome, infection-associated (D76.2).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 814 (Reticuloendothelial and Immunity Disorders with MCC, relative weight 2.0729), DRG 815 (Reticuloendothelial and Immunity Disorders with CC, relative weight 1.0444) and DRG 816 (Reticuloendothelial and Immunity Disorders without CC/MCC, relative weight 0.7381), in MDC 16 (Diseases and Disorders of the Blood and Blood Forming Organs and Immunological Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

More specific histiocytosis codes (official index)

  • acute differentiated progressive C96.0 — Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
  • Langerhans' cell NEC C96.6 — Unifocal Langerhans-cell histiocytosis
  • multifocal X
  • multisystemic (disseminated) C96.0 — Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
  • unisystemic C96.5 — Multifocal and unisystemic Langerhans-cell histiocytosis
  • pulmonary, adult (adult PLCH) J84.82 — Adult pulmonary Langerhans cell histiocytosis
  • unifocal (X) C96.6 — Unifocal Langerhans-cell histiocytosis
  • lipid, lipoid D76.3 — Other histiocytosis syndromes
  • essential E75.29 — Other sphingolipidosis
  • malignant C96.A — Histiocytic sarcoma
  • mononuclear phagocytes NEC D76.1 — Hemophagocytic lymphohistiocytosis
  • Langerhans' cells C96.6 — Unifocal Langerhans-cell histiocytosis
  • non-Langerhans cell D76.3 — Other histiocytosis syndromes
  • polyostotic sclerosing D76.3 — Other histiocytosis syndromes
  • sinus, with massive lymphadenopathy D76.3 — Other histiocytosis syndromes
  • syndrome NEC D76.3 — Other histiocytosis syndromes
  • X NEC C96.6 — Unifocal Langerhans-cell histiocytosis
  • acute (progressive) C96.0 — Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
  • chronic C96.6 — Unifocal Langerhans-cell histiocytosis
  • multifocal C96.5 — Multifocal and unisystemic Langerhans-cell histiocytosis
  • multisystemic C96.0 — Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis
  • unifocal C96.6 — Unifocal Langerhans-cell histiocytosis

Common questions about histiocytosis ICD-10 codes

What is the ICD-10 code for histiocytosis?

D76.3 — Other histiocytosis syndromes. More specific codes apply when the documentation supports them: C96.0, C96.6, C96.0, C96.5 and others below.

What is the ICD-10 code for acute differentiated progressive histiocytosis?

C96.0 — Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis.

What is the ICD-10 code for langerhans' cell nec histiocytosis?

C96.6 — Unifocal Langerhans-cell histiocytosis.

What is the ICD-10 code for malignant histiocytosis?

C96.A — Histiocytic sarcoma.

What is the ICD-10 code for mononuclear phagocytes nec histiocytosis?

D76.1 — Hemophagocytic lymphohistiocytosis.

What is the ICD-10 code for non-langerhans cell histiocytosis?

D76.3 — Other histiocytosis syndromes.

What is the ICD-10 code for polyostotic sclerosing histiocytosis?

D76.3 — Other histiocytosis syndromes.

Is D76.3 billable?

Yes. D76.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can D76.3 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict D76.3 as a principal diagnosis.

Is D76.3 a CC or MCC?

D76.3 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.